|
1
|
Nijim S, Fajgenbaum DC. Identifying Castleman disease from non-clonal inflammatory causes of generalized lymphadenopathy. HEMATOLOGY. AMERICAN SOCIETY OF HEMATOLOGY. EDUCATION PROGRAM 2024; 2024:582-593. [PMID: 39644038 DOI: 10.1182/hematology.2024000582] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/09/2024]
Abstract
Idiopathic multicentric Castleman disease (iMCD) is a rare, life-threatening subtype of Castleman disease (CD), which describes a group of rare, polyclonal lymphoproliferative disorders that demonstrate characteristic histopathology and variable symptomatology. iMCD involves a cytokine storm that occurs due to an unknown cause. Rapid diagnosis is required to initiate appropriate, potentially life-saving therapy, but diagnosis is challenging and impeded by clinical overlap with a wide spectrum of inflammatory, neoplastic, and infectious causes of generalized lymphadenopathy. Diagnosis, which requires both consistent histopathologic and clinical criteria, can be further delayed in the absence of close collaboration between clinicians and pathologists. A multimodal assessment is necessary to effectively discriminate iMCD from overlapping diseases. In this review, we discuss a pragmatic approach to generalized lymphadenopathy and clinical, laboratory, and histopathological features that can aid with identifying iMCD. We discuss diagnostic barriers that impede appropriate recognition of disease features, diagnostic criteria, and evidence-based treatment recommendations that should be initiated immediately following diagnosis.
Collapse
Affiliation(s)
- Sally Nijim
- Raymond and Ruth Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA
| | - David C Fajgenbaum
- Raymond and Ruth Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA
| |
Collapse
|
|
2
|
Tănase I, Sarafoleanu CC, Cobzeanu BM, Georgescu AM, Busuioc CI, Iovănescu D, Badea FC. Castleman disease as the great mimicker: the role of complex morpho-pathological evaluation. ROMANIAN JOURNAL OF MORPHOLOGY AND EMBRYOLOGY = REVUE ROUMAINE DE MORPHOLOGIE ET EMBRYOLOGIE 2024; 65:575-581. [PMID: 39957018 PMCID: PMC11924898 DOI: 10.47162/rjme.65.4.03] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/18/2025]
Abstract
Castleman disease (CD) is a group of lymphoproliferative disorders characterized by abnormal enlargement of lymph nodes (LNs) and a wide range of symptoms. Only a few cases are found in the head and neck. Based on clinical presentation and evolution, the disease can be classified into two main subtypes: unicentric CD (UCD), characterized by the enlargement of a single LN or a single LN chain, and multicentric CD (MCD), characterized by the involvement of multiple LNs and regions, with more important systemic symptoms and a poor prognosis. CD is also known as "the great mimicker" and can be easily misdiagnosed, sharing similar clinical and imaging characteristics with other pathologies, mainly when it is found in the neck. A histopathological (HP) and immunohistochemical (IHC) correlation is essential in these cases to accurately diagnose and establish the proper treatment plan. In light of this, we present a rare case of a 15-year-old young female with a slowly expanding, isolated supraclavicular mass. An excisional biopsy was performed, and the results confirmed the presence of CD, hyaline-vascular type. As of this writing, there was no evidence of recurrence or new enlarged LNs. We also review CD's clinical and HP features and the diagnostic and treatment challenges it poses.
Collapse
Affiliation(s)
- Ionuţ Tănase
- Department of Laboratory Medicine, Victor Babeş Clinical Hospital of Infectious Diseases and Pneumophthisiology, Craiova; Doctoral School, University of Medicine and Pharmacy of Craiova, Romania; ; Department of Otorhinolaryngology, Medicover Hospital, Bucharest, Romania;
| | | | | | | | | | | | | |
Collapse
|
|
3
|
Rani D, Varshney A, Rastogi K. Cytological diagnosis of hyaline-vascular type of Castleman disease. Autops Case Rep 2024; 14:e2024519. [PMID: 39372070 PMCID: PMC11452081 DOI: 10.4322/acr.2024.519] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2024] [Accepted: 08/13/2024] [Indexed: 10/08/2024]
Abstract
Castleman disease (CD) is a rare, benign lymphoproliferative disorder, mostly involving the mediastinal lymph nodes, but can occur wherever lymphoid tissue is found. With only a few published case reports, there needs to be more literature on its cytological findings. We report the case of a 63-year-old female presenting with left upper cervical swelling. Fine needle aspiration cytology smears showed variably sized lymphoid follicles with diminished germinal centers, prominence of follicular dendritic cells, and capillaries traversing some of the follicles. The possibility of a hyaline-vascular type of Castleman disease was suggested. Histopathology confirmed the cytological diagnosis. The index case is being presented to discuss the cytological features of the CD along with its histological and immunohistochemical correlation.
Collapse
Affiliation(s)
- Deepa Rani
- Sarojini Naidu Medical College, Department of Pathology, Agra, Uttar Pradesh, India
| | - Anupam Varshney
- Muzaffarnagar Medical College, Department of Pathology, Uttar Pradesh, India
| | - Kanika Rastogi
- Atal Bihari Vajpayee Institute of Medical Sciences & Dr Ram Manohar Lohia Hospital, Department of Pathology, New Delhi, India
| |
Collapse
|
|
4
|
AlSheikh S, Altoijry A, Al-Mubarak H, Alsallum OD, Alakeel F, Alanezi T. A rare presentation of unicentric Castleman's disease in the thigh: A case report and review of literature. World J Clin Cases 2024; 12:4003-4009. [PMID: 38994289 PMCID: PMC11235453 DOI: 10.12998/wjcc.v12.i19.4003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/06/2024] [Revised: 05/08/2024] [Accepted: 05/21/2024] [Indexed: 06/29/2024] Open
Abstract
BACKGROUND Castleman's disease (CD) is a rare lymphoproliferative, emulating both benign and malignant diseases. The diagnosis of CD is formulated upon the combination of clinical and laboratory criteria and ultimately confirmed by histopathological assessment. Due to its rarity, CD presents a challenge in treatment selection, with available options encompassing surgery, chemotherapy, and autologous stem cell transplantation. However, studies suggest that surgical resection of the lesion is the most effective treatment modality, especially for unicentric CD (UCD). CASE SUMMARY Here, we describe the case of a 25-year-old woman who presented with painless left thigh swelling for 10 wk. She had been following a low-fat diet to lose weight and had normal laboratory results. Magnetic resonance imaging revealed a well-circumscribed, demarcated cystic lesion located in the left inguinal region with eccentrically positioned signal void vascular structures, measuring 4.3 cm × 3 cm × 3.2 cm, likely of lymphoid origin. The patient underwent surgical resection, and the final histopathology showed a vascular proliferation and hyalinization of the vessel walls, along with atretic germinal centers traversed by penetrating vessels, consistent with CD. The patient was discharged home one day after the procedure in good condition, with a follow-up appointment scheduled in our outpatient clinic. CONCLUSION Although surgical resection is the mainstay for UCD, a multidisciplinary approach is needed due the lack of specific diagnostic features and treatments.
Collapse
Affiliation(s)
- Sultan AlSheikh
- Division of Vascular Surgery, Department of Surgery, College of Medicine, King Saud University, Riyadh 11322, Saudi Arabia
| | - Abdulmajeed Altoijry
- Division of Vascular Surgery, Department of Surgery, College of Medicine, King Saud University, Riyadh 11322, Saudi Arabia
| | - Husain Al-Mubarak
- Division of Vascular Surgery, Department of Surgery, College of Medicine, King Saud University, Riyadh 11322, Saudi Arabia
| | - Ofays Dakkam Alsallum
- Division of Vascular Surgery, Department of Surgery, College of Medicine, King Saud University, Riyadh 11322, Saudi Arabia
- Division of Vascular Surgery, Department of Surgery, King Khalid Hospital Najran, Najran 11321, Saudi Arabia
| | - Fadi Alakeel
- Department of Pathology, College of Medicine, King Saud University, Riyadh 11322, Saudi Arabia
| | - Tariq Alanezi
- Division of Vascular Surgery, Department of Surgery, College of Medicine, King Saud University, Riyadh 11322, Saudi Arabia
| |
Collapse
|
|
5
|
Rodolfi S, Della-Torre E, Bongiovanni L, Mehta P, Fajgenbaum DC, Selmi C. Lymphadenopathy in the rheumatology practice: a pragmatic approach. Rheumatology (Oxford) 2024; 63:1484-1493. [PMID: 38109670 PMCID: PMC11147542 DOI: 10.1093/rheumatology/kead644] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2023] [Revised: 10/31/2023] [Accepted: 11/05/2023] [Indexed: 12/20/2023] Open
Abstract
Lymphadenopathy is a common clinical finding and diagnostic challenge within general medicine and rheumatology practice. It may represent a primary manifestation of an underlying immune-mediated disease or indicate an infectious or neoplastic complication requiring differing management. Evaluating lymphadenopathy is of particular relevance in rheumatology, given that lymph node enlargement is a common finding within the clinical spectrum of several well-known rheumatologic disorders including RA, SLE and SS. In addition, lymphadenopathy represents a hallmark manifestation of rare immunological diseases such as Castleman disease and IgG4-related disease that must be considered in the differential diagnosis because effective targeted treatments can now impact the prognosis of these conditions. In this review we present an overview of the clinical significance of lymphadenopathy in common and rare rheumatologic diseases and propose a practical approach to lymphadenopathy in the rheumatology practice. Differential diagnosis of Castleman disease and therapeutic options for this condition of increasing rheumatologic interest will be discussed in detail.
Collapse
Affiliation(s)
- Stefano Rodolfi
- Department of Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Milan, Italy
- Department of Biomedical Sciences, Humanitas University, Milan, Italy
| | - Emanuel Della-Torre
- Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy
- Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Milan, Italy
- IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Lucia Bongiovanni
- Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy
- Department of Haematopathology Diagnostic Area, Unit of Pathology, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Puja Mehta
- Division of Medicine, University College, Centre for Inflammation and Tissue Repair, UCL Respiratory, London
- Department of Rheumatology, University College London Hospital (UCLH), London, UK
| | - David C Fajgenbaum
- Department of Medicine, Division of Translational Medicine and Human Genetics, Center for Cytokine Storm Treatment and Laboratory, Philadelphia, PA, USA
| | - Carlo Selmi
- Department of Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Milan, Italy
- Department of Biomedical Sciences, Humanitas University, Milan, Italy
| |
Collapse
|
|
6
|
Patel R, Patel D, Onyechi A, Shaikh S, Patel M, Patel Z. Castleman disease- demographics, associations, and outcomes: an analysis of adult 791 cases. Ann Hematol 2024; 103:1255-1260. [PMID: 38040860 DOI: 10.1007/s00277-023-05574-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2023] [Accepted: 11/29/2023] [Indexed: 12/03/2023]
Abstract
BACKGROUND Castleman disease (CD), also known as angiofollicular lymph node hyperplasia or large lymph node hyperplasia, is a rare medical condition. Despite its rarity, it exhibits diverse clinical presentations and outcomes, which pose challenges for comprehensive understanding and management. This study aims to shed light on the demographics, associations, and outcomes of CD by conducting a retrospective analysis. METHODS The National Inpatient Sample (US) was used to identify patients with the diagnosis of Castleman disease using ICD-10 diagnosis code D47.Z2, during the years 2016-2019. Data was collected on demographics, associated diagnoses, treatments and outcomes. Data analysis was performed using STATA Version 17, College Station, TX: Stata Corp LLC. RESULTS Our study identified 791 hospitalizations involving adult CD patients. The mean age of these patients was 52.4 years, with a male predominance (56.1%). Whites comprised the largest racial group affected (50.1%). Most patients were covered by Medicare (39.6%). The majority received treatment in urban teaching hospitals (84.0%) and large-bed size facilities (62.5%). In-hospital mortality was low at 2.8%, with an average length of stay of 7.5 days and average total charges of $109,308. Common associations included acute kidney injury (27.0%), congestive heart failure (17.1%), sepsis (16.4%), and acute respiratory failure (12.6%). Hematological and lymphatic associations featured anemia (47.5%), thrombocytopenia (12.2%), and other conditions. Red blood cell transfusions were administered to 11.1% of patients. CONCLUSION This study contributes valuable insights into CD, a rare and clinically heterogeneous disease. It underscores the importance of recognizing its associations and complications. Additionally, it highlights the need for further research and improved diagnostic and treatment guidelines to address the complexity of this condition.
Collapse
Affiliation(s)
- Rushin Patel
- Department of Internal Medicine, Community Hospital of San Bernardino, 805 Medical Center Drive, San Bernardino, CA, 92411, USA.
| | - Darshil Patel
- Clinical Research Program, Graduate College, Rush University, Chicago, IL, USA
| | - Afoma Onyechi
- Department of Internal Medicine, SSM Health St. Mary's Hospital, St Louis, MO, USA
| | - Safia Shaikh
- Department of Internal Medicine, Washington University, St Louis, MO, USA
| | - Mrunal Patel
- Department of Internal Medicine, Trumbull Regional Medical Center, Warren, OH, USA
| | - Zalak Patel
- Department of Internal Medicine, University of California Riverside, Riverside, CA, USA
| |
Collapse
|
|
7
|
Chabchoub I, Salah RB, Kallel R, Snoussi M, Frikha F, Marzouk S, Boudawara TS, Bahloul Z. Clinical features and outcomes in patients with human immunodeficiency virus-negative, Castleman's disease: a single medical center study in Tunisia. ROMANIAN JOURNAL OF INTERNAL MEDICINE = REVUE ROUMAINE DE MEDECINE INTERNE 2024; 62:20-32. [PMID: 37948573 DOI: 10.2478/rjim-2023-0028] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/04/2023] [Indexed: 11/12/2023]
Abstract
INTRODUCTION Castleman's disease (CD), known as angiofollicular lymph node hyperplasia, is an uncommon condition. The two most common histological subtypes are hyaline vascular and plasma cell. We performed a retrospective analysis to define the clinic-pathological features and survival of CD, which is quite rare focusing on the particularities of our series with a review of the recent literature. METHODS This is a retrospective study conducted in the department of internal medicine of Hedi Chaker hospital in Sfax, Tunisia over 25 years. The disease was histologically confirmed in all patients. For each file, we collected a set of data by filling in a pre-designed form. RESULTS 18 patients were included. There were 8 men and 10 women with a mean age of 42.8 years. CD was monocentric in 5 cases (28%) and multicentric in 13 cases (72%). Clinically, peripheral adenopathy was present in 77.7% of patients and deep adenopathy in 72.2%. Systemic signs were found in 13 patients, including general condition (4.4%), fever (16.6%), serositis (27.7%), and skin involvement (33.3%). A biological inflammatory syndrome accompanied the clinical picture in 66% of patients. Abnormalities in the blood count were found in 12 cases (66%), with anemia in 11 cases, thrombocytosis in 3 cases, and hypereosinophilia in 3 cases. Cutaneous Kaposi's sarcoma was associated with Castleman's disease in 2 cases, Hodgkin's lymphoma, angioimmunoblastic T-cell lymphoma, and lymph node T-cell lymphoma were found in 1 case respectively. 3 of the patients had associated connective tissue diseases such as Sjögren's syndrome in 2 cases and rheumatoid arthritis in 1 case. HHV8 serology was positive in 1 case with a multicentric plasma cell form. Histologically, the plasma cell form represented 50% of cases, hyaline-vascular (39% of cases), and mixed (11% of cases). Therapeutically, high-dose corticosteroid therapy was initiated in 13 cases. As a second-line treatment, MOPP chemotherapy was used in 1 case due to transformation into Hodgkin's lymphoma, and biotherapy (rituximab) was used in 2 cases in the multicentric form. Surgical removal of superficial adenopathy was performed in 2 patients with monocentric CD. CONCLUSION : Castleman's disease (CD) is a non-malignant lymphoproliferation of localized or multicentric form with a wide and heterogeneous clinical spectrum. Diagnosis can be difficult due to the lack of clinical and radiological specificity. Management depends on the clinical form involving surgical and/or medical management.
Collapse
Affiliation(s)
- Imen Chabchoub
- Internal Medicine department, Hedi Chaker Hopsital, University of Sfax, Faculty of Medicine 3029 Sfax, Tunisia
| | - Raida Ben Salah
- Internal Medicine department, Hedi Chaker Hopsital, University of Sfax, Faculty of Medicine 3029 Sfax, Tunisia
| | - Rim Kallel
- Anatomopathology laboratory, Habib Bourguiba Hospital, Sfax, Tunisia
| | - Mouna Snoussi
- Internal Medicine department, Hedi Chaker Hopsital, University of Sfax, Faculty of Medicine 3029 Sfax, Tunisia
| | - Feten Frikha
- Internal Medicine department, Hedi Chaker Hopsital, University of Sfax, Faculty of Medicine 3029 Sfax, Tunisia
| | - Sameh Marzouk
- Internal Medicine department, Hedi Chaker Hopsital, University of Sfax, Faculty of Medicine 3029 Sfax, Tunisia
| | | | - Zouhir Bahloul
- Internal Medicine department, Hedi Chaker Hopsital, University of Sfax, Faculty of Medicine 3029 Sfax, Tunisia
| |
Collapse
|
|
8
|
Sheth RK, Kumar P, Vishnurag A, Choudhary D, Pushparaj J. A Googly in the Neck-Castleman Disease. Indian J Otolaryngol Head Neck Surg 2024; 76:1275-1278. [PMID: 38440555 PMCID: PMC10908754 DOI: 10.1007/s12070-023-04266-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/28/2023] [Accepted: 10/05/2023] [Indexed: 03/06/2024] Open
Abstract
Lymphoproliferative disorders comprise of a group of heterogenous diseases characterised by uncontrolled production of lymphocytes.Castleman disease (CD) is one such disease and comprises of a group of rare disorders that involve enlarged lymph nodes and a broad range of inflammatory symptoms and laboratory abnormalities [1]. We came across young girl who presented to us in the out patient department with this disease After ruling out other pathologies and confirming the diagnosis by doing a tissue biopsy the girl was treated surgically and is disease free till date.
Collapse
Affiliation(s)
- Ritu K Sheth
- Department of ENT, Seth V.C Gandhi & M. A. Vora Municipal General Hospital, Mumbai, India
| | - Purnima Kumar
- Department of ENT, Seth V.C Gandhi & M. A. Vora Municipal General Hospital, Mumbai, India
| | - Athira Vishnurag
- Department of ENT, Seth V.C Gandhi & M. A. Vora Municipal General Hospital, Mumbai, India
| | - Deepika Choudhary
- Department of ENT, Seth V.C Gandhi & M. A. Vora Municipal General Hospital, Mumbai, India
| | - Jansimary Pushparaj
- Department of ENT, Seth V.C Gandhi & M. A. Vora Municipal General Hospital, Mumbai, India
| |
Collapse
|
|
9
|
Sumiyoshi R, Koga T, Fukui S, Furukawa K, Momoki M, Ichinose K, Yano S, Kawakami A. Exploring the role of insulin-like growth factor binding protein-1 in identifying idiopathic multicentric Castleman's disease types: Implications for the mTOR signaling pathway. Clin Immunol 2023; 256:109798. [PMID: 37778714 DOI: 10.1016/j.clim.2023.109798] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2023] [Accepted: 09/27/2023] [Indexed: 10/03/2023]
Abstract
OBJECTIVE To determine the molecular differences between iMCD-thrombocytopenia, anasarca, fevers, reticulin myelofibrosis, organomegaly (TAFRO), and iMCD-not otherwise specified (NOS). METHODS CD4-positive T cells were isolated from two iMCD-TAFRO and two iMCD-NOS patients for RNA sequencing comparison. Serum proteins of two iMCD-TAFRO and four iMCD-NOS patients were comprehensively analyzed to identify pathogenesis-associated proteins. IGFBP-1 protein, extracted from serum analysis, was compared to healthy controls, iMCD, systemic lupus erythematosus, and rheumatoid arthritis patients. RESULTS RNA sequencing of CD4-positive T cells revealed enhanced mTOR-related signaling in iMCD-TAFRO compared to iMCD-NOS. Comprehensive serum analysis found IGFBP-1 linked to iMCD pathogenesis, significantly higher in iMCD-TAFRO. This protein may be elevated in patients with iMCD caused by an enhanced mTOR pathway. CONCLUSION The mTOR pathway is suggested to be activated in iMCD-TAFRO compared to iMCD-NOS, which may elevate the protein IGFBP-1. This protein may be a biomarker to distinguish iMCD-TAFRO from iMCD-NOS.
Collapse
Affiliation(s)
- Remi Sumiyoshi
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan; Clincal Research Center, Nagasaki University Hospital, Nagasaki, Japan
| | - Tomohiro Koga
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
| | - Shoichi Fukui
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
| | - Kaori Furukawa
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
| | - Mamiko Momoki
- Division of Clinical Oncology & Hematology, Department of Internal Medicine, The Jikei University Kashiwa Hospital, Chiba, Japan
| | - Kunihiro Ichinose
- Department of Rheumatology, Shimane University Faculty of Medicine, Izumo, Japan
| | - Shingo Yano
- Division of Clinical Oncology & Hematology, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan
| | - Atsushi Kawakami
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
| |
Collapse
|
|
10
|
Abraham SS, Narayanan G, Thambi SM, Vasudevan JA, Joy Philip DS, Purushothaman PN, Nair SG, Nair R. Castleman disease: Experience from a single institution. MEDICINE INTERNATIONAL 2023; 3:56. [PMID: 37927353 PMCID: PMC10620848 DOI: 10.3892/mi.2023.116] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 05/22/2023] [Accepted: 10/02/2023] [Indexed: 11/07/2023]
Abstract
Castleman disease (CD) describes a group of rare heterogeneous lymphoproliferative disorders characterized by enlarged hyperplastic lymph nodes. It is classified into unicentric CD (UCD) and multicentric CD (MCD). The present retrospective study examined the data of 11 patients with CD diagnosed and treated at a tertiary cancer center from 2017 to 2022. The median age of the study group was 41 years (range, 24 to 68 years). There were 8 males and 3 females. In total, 7 patients were diagnosed with UCD and 4 patients with MCD. The hyaline-vascular variant was the most common histology in both UCD and MCD. Among the 7 patients with UCD, 5 patients underwent excision, 1 patient underwent debulking followed by radiotherapy and 1 patient received single agent rituximab. Of the patients with UCD, 6 had a complete response (CR) and 1 patient had a partial response (PR). All 4 patients with MCD received systemic treatment, which included single agent rituximab (2 patients), rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (RCHOP) (1 patient) and CHOP (1 patient). Among the patients with MCD, 1 patient attained a CR, 2 patients had a PR and 1 patient succumbed. The 3-year survival rate for the study population was 91%. In summary, CD is a rare disease occurring in immunodeficient patients. UCD is more common and is associated with better outcomes. Surgery is the mainstay of management in UCD whereas MCD requires combination chemotherapy.
Collapse
Affiliation(s)
- Sherry S. Abraham
- Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala 695011, India
| | - Geetha Narayanan
- Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala 695011, India
| | | | | | - Deepa Susan Joy Philip
- Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala 695011, India
| | - Prakash N. Purushothaman
- Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala 695011, India
| | - Sreejith G. Nair
- Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala 695011, India
| | - Rekha Nair
- Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala 695011, India
| |
Collapse
|
|
11
|
Horikawa D, Shimazaki R, Manabe K, Ichimura K, Ishibashi K, Fukasaku Y, Ishikawa T, Koshizuka Y, Shibaki T, Yanagida N, Akabane H, Yokoo H, Sumi Y. Hyaline-vascular type unicentric Castleman disease with dysplastic follicular dendritic cell proliferative lesions: a case report. J Surg Case Rep 2023; 2023:rjad536. [PMID: 37781046 PMCID: PMC10539102 DOI: 10.1093/jscr/rjad536] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2023] [Accepted: 08/30/2023] [Indexed: 10/03/2023] Open
Abstract
Castleman disease (CD) is a rare lymphoproliferative disease. Hyaline-vascular type unicentric CD has a good prognosis if completely resected during surgery. However, follicular dendritic cell proliferative lesions have the potential for recurrence and metastasis. A 22-year-old man was referred to our hospital with the chief complaint of nausea and vomiting. These symptoms were caused by a right mesocolonic tumor pushing the duodenum. The patient underwent laparoscopic tumorectomy and complete surgical excision. The postoperative course was uneventful, with no complications. Pathological examination confirmed that the tumor was an enlarged lymph node, typical of hyaline vascular-type CD; however, follicular dendritic cell proliferative lesions were noted. We report a rare case of hyaline-vascular-type CD with follicular dendritic cell proliferative lesions associated with malignancy, as limited case reports exist on this particular disease.
Collapse
Affiliation(s)
- Daisuke Horikawa
- Department of Surgery, Furano Kyokai Hospital, Furano, Hokkaido 076-8765, Japan
| | - Ryotaro Shimazaki
- Department of Surgery, Asahikawa Kosei Hospital, Asahikawa, Hokkaido 078-8211, Japan
| | - Kazuya Manabe
- Department of Surgery, Asahikawa Kosei Hospital, Asahikawa, Hokkaido 078-8211, Japan
| | - Kentaro Ichimura
- Department of Surgery, Asahikawa Kosei Hospital, Asahikawa, Hokkaido 078-8211, Japan
| | - Kei Ishibashi
- Department of Surgery, Asahikawa Kosei Hospital, Asahikawa, Hokkaido 078-8211, Japan
| | - Yasutomo Fukasaku
- Department of Surgery, Asahikawa Kosei Hospital, Asahikawa, Hokkaido 078-8211, Japan
| | - Takahisa Ishikawa
- Department of Surgery, Asahikawa Kosei Hospital, Asahikawa, Hokkaido 078-8211, Japan
| | - Yasuyuki Koshizuka
- Department of Surgery, Asahikawa Kosei Hospital, Asahikawa, Hokkaido 078-8211, Japan
| | - Taiichiro Shibaki
- Department of Surgery, Asahikawa Kosei Hospital, Asahikawa, Hokkaido 078-8211, Japan
| | - Naoyuki Yanagida
- Department of Surgery, Asahikawa Kosei Hospital, Asahikawa, Hokkaido 078-8211, Japan
| | - Hiromitsu Akabane
- Department of Surgery, Asahikawa Kosei Hospital, Asahikawa, Hokkaido 078-8211, Japan
| | - Hideki Yokoo
- Department of Hepatobiliary, Pancreatic and Transplant Surgery, Asahikawa Medical University, Asahikawa, Hokkaido 078-8510, Japan
| | - Yasuo Sumi
- Department of Digestive Surgery, Asahikawa Medical University, Asahikawa, Hokkaido 078-8510, Japan
| |
Collapse
|
|
12
|
Zhang L, Dong YJ, Peng HL, Li H, Zhang MZ, Wang HH, Liu QH, Su LP, Zhong LY, Wu WJ, Huang L, Yan XJ, Fan L, Tang WJ, Li ZL, Bi LT, Li Y, Gao GX, Gao L, Liu TB, Wei YQ, Liu Y, Yu L, Zhou H, Sun CY, Qian WB, Zou DH, Zhang HL, Ding KY, Wang XB, Bai O, Huang WR, Chen B, Yang L, Song J, Gao D, Chen T, Luo J, Wang SY, Ma LM, Fajgenbaum DC, Li J. A national, multicenter, retrospective study of Castleman disease in China implementing CDCN criteria. THE LANCET REGIONAL HEALTH. WESTERN PACIFIC 2023; 34:100720. [PMID: 37283978 PMCID: PMC10240357 DOI: 10.1016/j.lanwpc.2023.100720] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/04/2022] [Revised: 02/07/2023] [Accepted: 02/08/2023] [Indexed: 06/08/2023]
Abstract
Background Castleman disease (CD) is a group of rare and heterogenous lymphoproliferative disorders including unicentric CD (UCD), human herpesvirus-8(HHV-8)-associated multicentric CD (HHV8-MCD), and HHV-8-negative/idiopathic multicentric CD (iMCD). Knowledge of CD mainly comes from case series or retrospective studies, but the inclusion criteria of these studies vary because the Castleman Disease Collaborative Network (CDCN) diagnostic criteria for iMCD and UCD were not available until 2017 and 2020, respectively. Further, these criteria and guidelines have not been systematically evaluated. Methods In this national, multicenter, retrospective study implementing CDCN criteria, we enrolled 1634 CD patients (UCD, n = 903; MCD, n = 731) from 2000 to 2021 at 40 Chinese institutions to depict clinical features, treatment options, and prognostic factors of CD. Findings Among UCD, there were 162 (17.9%) patients with an MCD-like inflammatory state. Among MCD, there were 12 HHV8-MCD patients and 719 HHV-8-negative MCD patients, which included 139 asymptomatic MCD (aMCD) and 580 iMCD meeting clinical criteria. Of 580 iMCD patients, 41 (7.1%) met iMCD-TAFRO criteria, the others were iMCD-NOS. iMCD-NOS were further divided into iMCD-IPL (n = 97) and iMCD-NOS without IPL (n = 442). Among iMCD patients with first-line treatment data, a trend from pulse combination chemotherapy toward continuous treatment was observed. Survival analysis revealed significant differences between subtypes and severe iMCD (HR = 3.747; 95% CI: 2.112-6.649, p < 0.001) had worse outcome. Interpretation This study depicts a broad picture of CD, treatment options and survival information in China and validates the association between the CDCN's definition of severe iMCD and worse outcomes, requiring more intensive treatment. Fundings Beijing Municipal Commission of Science and Technology, CAMS Innovation Fund and National High Level Hospital Clinical Research Funding.
Collapse
Affiliation(s)
- Lu Zhang
- Department of Haematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yu-jun Dong
- Department of Haematology, Peking University First Hospital, Beijing, China
| | - Hong-ling Peng
- Department of Haematology, Second Xiang-ya Hospital, Central South University, Changsha, China
| | - Hao Li
- Department of Haematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Ming-zhi Zhang
- Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Hui-han Wang
- Department of Haematology, Sheng Jing Hospital of China Medical University, Shenyang, China
| | - Qin-hua Liu
- Department of Haematology, The First Affiliated Hospital of Anhui Medical University, Hefei, China
| | - Li-ping Su
- Department of Haematology, Shanxi Province Cancer Hospital, Taiyuan, China
| | - Li-ye Zhong
- Department of Haematology, Guangdong Provincial People's Hospital/Guangdong Academy of Medical Sciences, Guangzhou, China
| | - Wen-jun Wu
- Bone Marrow Transplantation Center, The First Affiliated Hospital of Zhejiang Medical College, Zhejiang University, Hangzhou, China
| | - Liang Huang
- Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Xiao-jing Yan
- Department of Haematology, First Hospital of China Medical University, Shenyang, China
| | - Lei Fan
- Department of Haematology, Jiangsu Province Hospital and Nanjing Medical University First Affiliated Hospital, Nanjing, China
| | - Wen-jiao Tang
- Department of Haematology, West China Hospital Sichuan University, Chengdu, China
| | - Zhen-ling Li
- Department of Haematology, China-Japan Friendship Hospital, Beijing, China
| | - Lin-tao Bi
- Department of Haematology and Oncology, China-Japan Union Hospital of Jilin University, Changchun, China
| | - Yan Li
- Department of Haematology, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, China
| | - Guang-xun Gao
- Department of Haematology, Xijing Hospital, Fourth Military Medical University, Xi'an, China
| | - Li Gao
- Department of Haematology, Xinqiao Hospital, Army Medical University, Chongqing, China
| | - Ting-bo Liu
- Department of Haematology, Fujian Medical University Union Hospital, Fuzhou, China
| | - Yong-qiang Wei
- Department of Haematology, Nanfang Hospital, Southern Medical University, Guangzhou, China
| | - Yao Liu
- Department of Haematology/Oncology, Chongqing University Cancer Hospital, Chongqing Cancer Hospital, Chongqing, China
| | - Li Yu
- Department of Haematology, The Second Affiliated Hospital of Nanchang University, Nanchang, China
| | - Hui Zhou
- Department of Lymphoma & Haematology, Hunan Cancer Hospital/The Affiliated Tumor Hospital of Xiangya Medical School, Central South University, Changsha, China
| | - Chun-yan Sun
- Institute of Haematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Wen-bin Qian
- Department of Haematology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China
| | - De-hui Zou
- Department of Lymphoma and Myeloma, State Key Laboratory of Experimental Haematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Haematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China
| | - Hui-lai Zhang
- Department of Lymphoma, Tianjin Medical University Cancer Institute and Hospital, Tianjin, China
| | - Kai-yang Ding
- Department of Haematology, The First Affiliated Hospital of USTC, Hefei, China
| | - Xiao-bo Wang
- Department of Haematology, The Second Hospital of Dalian Medical University, Dalian, China
| | - Ou Bai
- Department of Haematology, The First Hospital of Jilin University, Changchun, China
| | - Wen-rong Huang
- Department of Haematology, The Fifth Medical Center of Chinese PLA General Hospital, Beijing, China
| | - Bing Chen
- Department of Haematology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, China
| | - Lin Yang
- Department of Haematology, Second Hospital of Hebei Medical University, Shijiazhuang, China
| | - Jia Song
- Department of Haematology, Tianjin Medical University General Hospital, Tianjin, China
| | - Da Gao
- Department of Haematology, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, China
| | - Tong Chen
- Department of Haematology, Huashan Hospital, Fudan University, Shanghai, China
| | - Jun Luo
- Department of Haematology, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Shu-ye Wang
- Department of Haematology, The First Affiliated Hospital of Harbin Medical University, Harbin, China
| | - Liang-ming Ma
- Department of Haematology, Shanxi Bethune Hospital, Taiyuan, China
| | - David C. Fajgenbaum
- Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
| | - Jian Li
- Department of Haematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - China Castleman Disease Network (CCDN)
- Department of Haematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Department of Haematology, Peking University First Hospital, Beijing, China
- Department of Haematology, Second Xiang-ya Hospital, Central South University, Changsha, China
- Department of Haematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
- Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
- Department of Haematology, Sheng Jing Hospital of China Medical University, Shenyang, China
- Department of Haematology, The First Affiliated Hospital of Anhui Medical University, Hefei, China
- Department of Haematology, Shanxi Province Cancer Hospital, Taiyuan, China
- Department of Haematology, Guangdong Provincial People's Hospital/Guangdong Academy of Medical Sciences, Guangzhou, China
- Bone Marrow Transplantation Center, The First Affiliated Hospital of Zhejiang Medical College, Zhejiang University, Hangzhou, China
- Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Department of Haematology, First Hospital of China Medical University, Shenyang, China
- Department of Haematology, Jiangsu Province Hospital and Nanjing Medical University First Affiliated Hospital, Nanjing, China
- Department of Haematology, West China Hospital Sichuan University, Chengdu, China
- Department of Haematology, China-Japan Friendship Hospital, Beijing, China
- Department of Haematology and Oncology, China-Japan Union Hospital of Jilin University, Changchun, China
- Department of Haematology, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, China
- Department of Haematology, Xijing Hospital, Fourth Military Medical University, Xi'an, China
- Department of Haematology, Xinqiao Hospital, Army Medical University, Chongqing, China
- Department of Haematology, Fujian Medical University Union Hospital, Fuzhou, China
- Department of Haematology, Nanfang Hospital, Southern Medical University, Guangzhou, China
- Department of Haematology/Oncology, Chongqing University Cancer Hospital, Chongqing Cancer Hospital, Chongqing, China
- Department of Haematology, The Second Affiliated Hospital of Nanchang University, Nanchang, China
- Department of Lymphoma & Haematology, Hunan Cancer Hospital/The Affiliated Tumor Hospital of Xiangya Medical School, Central South University, Changsha, China
- Institute of Haematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Department of Haematology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China
- Department of Lymphoma and Myeloma, State Key Laboratory of Experimental Haematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Haematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China
- Department of Lymphoma, Tianjin Medical University Cancer Institute and Hospital, Tianjin, China
- Department of Haematology, The First Affiliated Hospital of USTC, Hefei, China
- Department of Haematology, The Second Hospital of Dalian Medical University, Dalian, China
- Department of Haematology, The First Hospital of Jilin University, Changchun, China
- Department of Haematology, The Fifth Medical Center of Chinese PLA General Hospital, Beijing, China
- Department of Haematology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, China
- Department of Haematology, Second Hospital of Hebei Medical University, Shijiazhuang, China
- Department of Haematology, Tianjin Medical University General Hospital, Tianjin, China
- Department of Haematology, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, China
- Department of Haematology, Huashan Hospital, Fudan University, Shanghai, China
- Department of Haematology, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
- Department of Haematology, The First Affiliated Hospital of Harbin Medical University, Harbin, China
- Department of Haematology, Shanxi Bethune Hospital, Taiyuan, China
- Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
| |
Collapse
|
|
13
|
Aldosari S, Altalhi B, Albisher N, Mohammed S, Alhazmi A, Alsobhi S. Castleman disease: a case report of an unusual adrenal incidentaloma and literature review. J Surg Case Rep 2023; 2023:rjad248. [PMID: 37220596 PMCID: PMC10200125 DOI: 10.1093/jscr/rjad248] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2023] [Accepted: 04/10/2023] [Indexed: 05/25/2023] Open
Abstract
Adrenal incidentaloma (AI) is an incidental detection of an adrenal mass on an image not performed for a suspected adrenal problem. AI has become a commonly encountered lesion that requires further investigations for evidence of hormonal hypersecretion or malignancy potential. According to guidelines, surgical intervention is the standard of care for unilateral AI. We report on a case of a 64-year-old female who presented with a nonfunctional adrenal mass associated with compressive symptoms, which was revealed to be a mixed hyaline vascular and plasma cell variant Castleman disease (CD) after surgical resection. Although hyaline vascular variant and plasma cell variant of CD has been identified in adrenal glands, this is the first report of a mixed hyaline vascular and plasma cell variant in an adrenal mass.
Collapse
Affiliation(s)
- Sarah Aldosari
- Correspondence address. Department of Surgery, King Faisal Specialist Hospital & Research Center, P.O. Box 3354, Riyadh 11211, Saudi Arabia. Tel: 966503565794; E-mail:
| | - Bassam Altalhi
- Department of Surgery, Almoosa Specialist Hospital, Alahsa, Saudi Arabia
| | - Nesreen Albisher
- Department of Surgery, Almoosa Specialist Hospital, Alahsa, Saudi Arabia
| | - Shamayel Mohammed
- Pathology & Laboratory Medicine Department, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
| | - Alaa Alhazmi
- Department of Surgery, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
| | - Saif Alsobhi
- Department of Surgery, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
| |
Collapse
|
|
14
|
Jumaat AF, Mohamad Yunus MR, Yong DJ, Md Zin RR, Mat Baki M. Multicentric Castleman Disease of the Supraglottis: A Surgeons' Dilemma. IRANIAN JOURNAL OF OTORHINOLARYNGOLOGY 2023; 35:101-108. [PMID: 37223400 PMCID: PMC10202163 DOI: 10.22038/ijorl.2023.57806.2995] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Figures] [Subscribe] [Scholar Register] [Received: 06/08/2021] [Accepted: 02/23/2022] [Indexed: 05/25/2023]
Abstract
Introduction An abnormal mass in the head and neck involving the supraglottic and cervical region offers a wide range of differential diagnoses. The pathology is either benign or malignant in nature. Castleman disease (CD) is an uncommon lymphoproliferative disorder characterised by hypervascular lymphoid hyperplasia and is classified into unicentric or multicentric disease. Histopathologically it is divided into hyaline vascular (HV), plasma cell (PC), and mixed cellularity variants. The multicentric disease is linked with PC and has the propensity to progress to lymphoma or Kaposi Sarcoma. Case Report We report a case of a 45-year-old gentleman who presented with a painless anterior neck swelling and left supraglottic mass for six months. Computed tomography (CT) contrast imaging demonstrated a homogenous enhancing lesion at the left supraglottic and the midline of the anterior neck with erosive changes of the thyroid cartilage. A surgical resection of the anterior neck mass was performed. The diagnosis of Castleman disease plasma cell variant was made by histopathologic evaluation. The patient remained well post-resection. Conclusion Supraglottic multicentric Castleman disease is the least expected diagnosis in this case. Unicentric disease is treated with surgery. However, limited studies are available in determining the effectiveness of surgery in multicentric diseases. The plasma cell variant requires a multidisciplinary and multimodal approach due to an inclination towards malignancy. Research is needed to determine the role of surgery in multicentric disease and to develop optimum guidelines for managing cases. To date, there is unsubstantial literature describing supraglottic multicentric disease.
Collapse
Affiliation(s)
- Anna Fariza Jumaat
- Department of Otorhinolaryngology Head and Neck Surgery, Faculty of Medicine, University Kebangsaan Malaysia Medical Centre, 56000 Kuala Lumpur, Malaysia.
| | - Mohd Razif Mohamad Yunus
- Department of Otorhinolaryngology Head and Neck Surgery, Faculty of Medicine, University Kebangsaan Malaysia Medical Centre, 56000 Kuala Lumpur, Malaysia.
| | - Doh Jeing Yong
- Department of Otorhinolaryngology Head and Neck Surgery, Faculty of Medicine, University Kebangsaan Malaysia Medical Centre, 56000 Kuala Lumpur, Malaysia.
| | - Reena Rahayu Md Zin
- Department of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Center, 56000 Kuala Lumpur, Malaysia
| | - Marina Mat Baki
- Department of Otorhinolaryngology Head and Neck Surgery, Faculty of Medicine, University Kebangsaan Malaysia Medical Centre, 56000 Kuala Lumpur, Malaysia.
| |
Collapse
|
|
15
|
Molacek J, Treska V, Skalicky T, Vodicka J, Ferda J, Ferdova E, Baxa J, Mach C, Jungova A, Michal M. Unicentric form of Castleman´s disease, pitfalls of diagnosis and surgical treatment. Front Oncol 2023; 13:1057683. [PMID: 36793610 PMCID: PMC9923952 DOI: 10.3389/fonc.2023.1057683] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/29/2022] [Accepted: 01/16/2023] [Indexed: 01/31/2023] Open
Abstract
Background Castleman´s disease is an extremely rare heterogenous lymphoproliferative pathology with a mostly benign behavior. It is a localized or generalized lymph node enlargement of an unknown aetiology. Unicentric form is typically a slow-growing solitary mass occurring mostly in the mediastinum, abdominal cavity, retroperitoneum, pelvis and neck. Aetiology and pathogenesis of CD is probably diverse, varying in different types of this heterogeneous disease. Materials and Methods Authors present a review of this issue based on their extensive experience. The aim is to summarize the crucial factors in the management of diagnostics and a surgical treatment of the unicentric form of Castleman´s disease. One of the key issues in the unicentric form is precise preoperative diagnostics and thus choosing the right surgical treatment strategy. Authors highlight pitfalls of the diagnosis and surgical treatment. Results All histological types such as a hyaline vascular type, plasmacytic type and a mixed type are presented as well as options of surgical and conservative treatment. Differential diagnosis and malignant potential is discussed. Conclusion Patients with Castleman´s disease should be treated in the high- volume centers, with a great experience in major surgical procedures as well as with preoperative imaging diagnostic techniques. Specialized pathologists and oncologists focusing on this issue are also absolutely necessary to avoid misdiagnosis. Only this complex approach can lead to excellent outcomes in patients with UCD.
Collapse
Affiliation(s)
- Jiri Molacek
- Faculty of Medicine in Pilsen, Charles University, Plzen, Czechia.,Department of Surgery, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia
| | - Vladislav Treska
- Department of Surgery, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia
| | - Tomas Skalicky
- Department of Surgery, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia
| | - Josef Vodicka
- Department of Surgery, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia
| | - Jiri Ferda
- Department of Imaging Methods, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia
| | - Eva Ferdova
- Department of Imaging Methods, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia
| | - Jan Baxa
- Department of Imaging Methods, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia
| | - Christiana Mach
- Department of Surgery, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czechia
| | - Alexandra Jungova
- Department of Hematology and Oncology, University Hospital in Pilsen, Plzen, Czechia
| | - Michael Michal
- Faculty of Medicine in Pilsen, Charles University, Plzen, Czechia
| |
Collapse
|
|
16
|
Sonoda K, Kaneko U, Hiura M, Kinoshita Y, Umezu H, Ito S, Saitoh A, Imai C. Short stature as an initial presenting presentation of unicentric Castleman disease in a child: A case report with long-term follow-up and a literature review. Mod Rheumatol Case Rep 2023; 7:261-266. [PMID: 35536586 DOI: 10.1093/mrcr/rxac034] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2022] [Revised: 04/02/2022] [Accepted: 04/11/2022] [Indexed: 06/14/2023]
Abstract
Short stature is a common clinical condition in paediatric outpatient clinics and is associated with various clinical conditions, ranging from normal variants to severe diseases. Short stature is known to be caused by chronic inflammatory conditions, in which over-produced inflammatory cytokines are reported to be involved in growth suppression. Castleman disease is a rare lymphoproliferative disorder known as a chronic inflammatory disease with overproduction of interleukin 6, which often causes systemic symptoms such as fever, fatigue, weight loss, and night sweats. Here, we report the case of a 10-year-old female diagnosed with unicentric Castleman disease, who presented with short stature as the sole clinical sign but lacked typical systemic symptoms of Castleman disease. An elevated serum C-reactive protein level led us to suspect a chronic inflammatory condition, and we found an intra-abdominal tumour that was histopathologically confirmed as Castleman disease. The tumour removal resulted in a steady catch-up in her height in the six years following the surgery. We also present a brief review of relevant literature on paediatric cases of Castleman disease associated with growth impairment. Clinicians should be aware that chronic inflammatory conditions can cause growth impairment, which may be a key clinical manifestation of such conditions.
Collapse
Affiliation(s)
- Kaori Sonoda
- Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
- Department of Pediatrics, Niigata Medical-Care Cooperative, Kido Hospital, Niigata, Japan
- Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan
| | - Utako Kaneko
- Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
| | - Makoto Hiura
- Department of Pediatrics, Niigata Medical-Care Cooperative, Kido Hospital, Niigata, Japan
| | - Yoshiaki Kinoshita
- Department of Pediatric Surgery, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
| | - Hajime Umezu
- Division of Pathology, Niigata University Medical and Dental Hospital, Niigata, Japan
| | - Shuichi Ito
- Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan
| | - Akihiko Saitoh
- Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
| | - Chihaya Imai
- Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
| |
Collapse
|
|
17
|
Fujiwara Y, Tanizawa Y, Shinoda K, Nagai T, Mamiya A, Aizawa S, Abe S, Kitano H. Unicentric plasma cell type of Castleman’s disease in the submandibular: A case report and literature review. JOURNAL OF ORAL AND MAXILLOFACIAL SURGERY, MEDICINE, AND PATHOLOGY 2023. [DOI: 10.1016/j.ajoms.2023.01.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/26/2023]
|
|
18
|
Phulware RH, Ramteke P, Yadav R, Iyer VK, Mallick S. Cytology of Castleman's disease (hyaline-vascular type) masquerading as Hodgkin's lymphoma. AMERICAN JOURNAL OF BLOOD RESEARCH 2022; 12:196-200. [PMID: 36742279 PMCID: PMC9890186] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 04/30/2022] [Accepted: 11/13/2022] [Indexed: 02/07/2023]
Abstract
Castleman disease (CD) is a rare benign disorder presents as a lymph nodal mass in mediastinum, cervical, axillary or abdomen. Due to the presence of dysplastic dendritic cell in a background mature lymphocyte and plasma cell, it mimics Hodgkin disease (HD). Synchronous and metachronous occurrence in HD and CD can also occur. An 11-year-old male presented with cervical lymphadenopathy (3.5 × 3.5 cm). Fine needle aspiration shows atypical binucleate cell in a background of small lymphocytes, a diagnosis of Hodgkin disease is suggested. Excisional biopsy showed classical features of Hyaline vascular Castleman disease. Careful cytological evaluation and clinical correlation is required for definitive diagnosis.
Collapse
Affiliation(s)
- Ravi Hari Phulware
- Department of Pathology, All India Institute of Medical ScienceRishikesh, India
| | - Prashant Ramteke
- Department of Pathology, All India Institute of Medical SciencesNew Delhi-110029, India
| | - Rajni Yadav
- Department of Pathology, All India Institute of Medical SciencesNew Delhi-110029, India
| | - Venkateswaran K Iyer
- Department of Pathology, All India Institute of Medical SciencesNew Delhi-110029, India
| | - Saumyaranjan Mallick
- Department of Pathology, All India Institute of Medical SciencesNew Delhi-110029, India
| |
Collapse
|
|
19
|
Viallard JF, Roriz M, Parrens M, Bonnotte B. Diagnostics différentiels de la maladie de Castleman. Rev Med Interne 2022; 43:10S17-10S25. [PMID: 36657939 DOI: 10.1016/s0248-8663(23)00021-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/19/2023]
Abstract
Clinicians are sometimes confronted with the diagnostic difficulties of the idiopathic form of Castleman's Disease (iMCD). As this review reports with demonstrative clinical cases, iMCD can mimic various serious systemic pathologies such as certain autoimmune diseases, Still's disease, POEMS syndrome, and malignant lymphoproliferations, sharing a very similar histology and identical symptoms. To make a diagnosis of iMCD, the clinician must eliminate all the pathologies mentioned above, but he must first think of it and evoke this diagnosis of rare disease before the first symptoms but also know how to evoke this diagnosis again even after several years of evolution of a disease like those mentioned above whose evolution is not favorable. © 2022 Published by Elsevier Masson SAS on behalf of Société nationale française de médecine interne (SNFMI).
Collapse
Affiliation(s)
- J F Viallard
- Service de médecine interne et maladies infectieuses, hôpital Haut-Lévêque, CHU de Bordeaux, 5, avenue de Magellan, 33604 Pessac, France.
| | - M Roriz
- Service de médecine interne et maladies infectieuses, hôpital d'Agen, Agen, France
| | - M Parrens
- Service d'anatomopathologie, hôpital Haut-Lévêque, CHU de Bordeaux, 5, avenue de Magellan, 33604 Pessac
| | - B Bonnotte
- Service de médecine interne et immunologie clinique, CHU de Dijon, Dijon, France
| |
Collapse
|
|
20
|
Yu T, Cai QQ, Zhai QL, Li L, Fang X, Li J, Sun R, Yang H, Wang Z, Qian W, Xu-Monette ZY, Young KH, Yu L. Analysis of albumin as a prognostic factor in HHV-8/HIV-negative Castleman disease from a multicenter study. Leuk Lymphoma 2022; 63:3082-3091. [PMID: 36074798 DOI: 10.1080/10428194.2022.2118528] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/11/2023]
Abstract
As a rare lymphoproliferative disorder, many patients with HHV-8/HIV-negative Castleman disease (CD) have hypoalbuminemia. However, data is limited on whether hypoalbuminemia is an independent predictor of CD. We retrospectively collected data from 230 patients diagnosed at 12 medical centers in China and the U.S. Different classifications included 147 patients with unicentric CD (UCD) and 83 with idiopathic multicentric CD (iMCD). Adjusted smooth curve fitting showed that the relationship between albumin and all-cause death of patients with CD and iMCD was linear. Cox proportional hazards regression modeling showed a negative association between the risk of death and albumin level (hazard ratio [HR]: 0.84; 95% CI, 0.76, 0.93). Using the Kaplan-Meier method, we determined that hypoproteinemia was a risk factor for poorer prognosis in patients with CD, UCD, and iMCD. Albumin was independently and negatively associated with the risk of death in CD patients, especially those with iMCD.
Collapse
Affiliation(s)
- Tiantian Yu
- Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang University, Nanchang, China
| | - Qing-Qing Cai
- State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Sun Yat-sen University Cancer Center, Guangzhou, China
| | - Qiong-Li Zhai
- Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, Tianjin, China
| | - Ling Li
- Department of Medical Oncology, Zhenzhou University Cancer Center, Zhenzhou, China
| | - Xiaosheng Fang
- Department of Hematology and Oncology, Shandong Cancer Hospital, Shandong, China
| | - Jianyong Li
- Department of Hematology and Oncology, Jiangsu Province Hospital, Nanjing University, Nanjing, China
| | - Ruifang Sun
- Department of Pathology, Shanxi Cancer Hospital, Shanxi, China
| | - Hanjin Yang
- Department of Pathology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Zhaoming Wang
- Department of Pathology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Wenbian Qian
- Department of Hematology and Oncology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Zijun Y Xu-Monette
- Division of Hematopathology and Department of Pathology, Duke University Medical Center, Durham, NC, USA
| | - Ken H Young
- Division of Hematopathology and Department of Pathology, Duke University Medical Center, Durham, NC, USA.,Duke University Cancer Center, Durham, NC, USA
| | - Li Yu
- Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang University, Nanchang, China
| |
Collapse
|
|
21
|
Krokenberger M, Schwamborn K, Strassen U. Prevalence of Castleman's disease in patients suffering from cervical lymphadenopathy. Laryngoscope Investig Otolaryngol 2022; 7:1430-1435. [PMID: 36258858 PMCID: PMC9575065 DOI: 10.1002/lio2.891] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2022] [Revised: 07/29/2022] [Accepted: 07/30/2022] [Indexed: 11/07/2022] Open
Abstract
Objectives To determine the prevalence of Castleman's disease (unicentric/idiopathic multicentric CD) in a retrospective cohort according to the newly defined international diagnostic criteria in patients, who underwent a lymph node removal at a tertiary care university hospital over a period of 10 years. Study design Retrospective chart review. Material and methods All Patients with cervical lymphadenopathy coded by ICD-10-CM with "I88.9," "R59.0," or "D47.Z2" between January 2010 and December 2020 and who underwent a lymph node extirpation were identified. In cases who met the diagnostic criteria for a potential unicentric or idiopathic multicentric CD (iMCD) diagnosis, the lymph node tissue was re-evaluated by a pathologist. Results A total of 714 patients with cervical lymphadenopathy were included into this single-center retrospective study. After exclusion of patients with diseases that may mimic iMCD and cases for which material to perform histological re-evaluation was lacking, a subset of 75 patients with "nonspecific lymphadenitis" or "reactive hyperplasia of lymph node" was identified, who underwent a renewed histopathological examination. One case fulfilled both the major and minor criteria of an iMCD diagnosis, and further 15 cases matched the histological criterion of an iMCD diagnosis (one of the two major diagnostic criteria), so that a UCD diagnosis according to the new criteria could be accepted. Conclusion In this cohort, the subsequent application of the new diagnostic criteria led to further cases of CD (1.9% compared to 0.1% before) being recognized. Although incidence and prevalence of UCD and iMCD are low, clinicians should keep in mind this differential diagnosis as effective therapies are available. Level of Evidence 4.
Collapse
Affiliation(s)
- Michael Krokenberger
- Department of Otolaryngology–Head & Neck SurgeryKlinikum rechts der Isar, Technical University of Munich (TUM)MunichGermany
| | - Kristina Schwamborn
- Institute of Pathology, Klinikum rechts der Isar, Technical University of Munich (TUM)MunichGermany
| | - Ulrich Strassen
- Department of Otolaryngology–Head & Neck SurgeryKlinikum rechts der Isar, Technical University of Munich (TUM)MunichGermany
| |
Collapse
|
|
22
|
Liu W, Cai Q, Yu T, Strati P, Hagemeister FB, Zhai Q, Zhang M, Li L, Fang X, Li J, Sun R, Zhang S, Yang H, Wang Z, Qian W, Iwaki N, Sato Y, Oksenhendler E, Xu-Monette ZY, Young KH, Yu L. Clinical characteristics and outcomes of Castleman disease: a multicenter Consortium study of 428 patients with 15-year follow-up. Am J Cancer Res 2022; 12:4227-4240. [PMID: 36225639 PMCID: PMC9548017] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/07/2022] [Accepted: 06/30/2022] [Indexed: 06/16/2023] Open
Abstract
Castleman disease (CD) has been reported as a group of poorly understood lymphoproliferative disorders, including unicentric CD (UCD) and idiopathic multicentric CD (iMCD) which are human immunodeficiency virus (HIV) negative and human herpes virus 8 (HHV-8) negative. The clinical and independent prognostic factors of CD remain poorly elucidated. We retrospectively collected the clinical information of 428 patients with HIV and HHV-8 negative CD from 12 large medical centers with 15-year follow-up. We analyzed the clinicopathologic features of 428 patients (248 with UCD and 180 with iMCD) with a median age of 41 years. The histology subtypes were hyaline-vascular (HV) histopathology for 215 patients (56.58%) and plasmacytic (PC) histopathology for 165 patients (43.42%). Most patients with UCD underwent surgical excision, whereas the treatment strategies of patients with iMCD were heterogeneous. The outcome for patients with UCD was better than that for patients with iMCD, 5-year overall survival (OS) rates were 95% and 74%, respectively. In further analysis, a multivariate analysis using a Cox regression model revealed that PC subtype, hepatomegaly and/or splenomegaly, hemoglobin ≤ 80 g/L, and albumin ≤ 30 g/L were independent prognostic factors of CD for OS. The model of iMCD revealed that age > 60 years, hepatomegaly and/or splenomegaly, and hemoglobin ≤ 80 g/L were independent risk factors. In UCD, single-factor analysis identified two significant risk factors: hemoglobin ≤ 100 g/L and albumin ≤ 30 g/L. Our study emphasizes the distinction of clinical characteristics between UCD and iMCD. The importance of poor risk factors of different clinical classifications may direct more precise and appropriate treatment strategies.
Collapse
Affiliation(s)
- Wanying Liu
- Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang UniversityNanChang, China
| | - Qingqing Cai
- State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Sun Yat-sen University Cancer CenterGuangzhou, China
| | - Tiantian Yu
- Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang UniversityNanChang, China
| | - Paolo Strati
- Department of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer CenterHouston, TX, USA
| | - Frederick B Hagemeister
- Department of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer CenterHouston, TX, USA
| | - Qiongli Zhai
- Department of Pathology, Tianjin Medical University Cancer Institute and HospitalTianjin, China
| | - Mingzhi Zhang
- Department of Medical Oncology, Zhengzhou University Cancer CenterZhengzhou, China
| | - Ling Li
- Department of Medical Oncology, Zhengzhou University Cancer CenterZhengzhou, China
| | - Xiaosheng Fang
- Department of Hematology and Oncology, Shandong Cancer HospitalShandong, China
| | - Jianyong Li
- Department of Hematology and Oncology, Jiangsu Province Hospital, Nanjing UniversityNanjing, China
| | - Ruifang Sun
- Department of Pathology, Shanxi Cancer HospitalShanxi, China
| | - Shanxiang Zhang
- Department of Pathology, Indiana University School of MedicineIndianapolis, IN, USA
| | - Hanjin Yang
- Department of Pathology, The First Affiliated Hospital, Zhejiang University School of MedicineHangzhou, China
| | - Zhaoming Wang
- Department of Pathology, The First Affiliated Hospital, Zhejiang University School of MedicineHangzhou, China
| | - Wenbian Qian
- Department of Hematology and Oncology, The Second Affiliated Hospital, Zhejiang University School of MedicineHangzhou, China
| | - Noriko Iwaki
- Department of Hematology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa UniversityKanazawa, Japan
| | - Yasuharu Sato
- Division of Pathophysiology, Okayama University Graduate School of Health SciencesOkayama, Japan
| | - Eric Oksenhendler
- Département d’Immunologie Clinique, Hôpital Saint-LouisParis, France
| | - Zijun Y Xu-Monette
- Division of Hematopathology and Department of Pathology, Duke University Medical CenterDurham, NC, USA
| | - Ken H Young
- Division of Hematopathology and Department of Pathology, Duke University Medical CenterDurham, NC, USA
- Duke University Cancer CenterDurham, NC, USA
| | - Li Yu
- Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang UniversityNanChang, China
| |
Collapse
|
|
23
|
Abstract
Castleman's disease – also known as giant lymph node hyperplasia – is a rare pathology, poorly understood. Described for the first time in 1954, it is considered a lymphoproliferative disorder that manifests without any other obvious symptoms and may be confused with other causes of lymph nodal enlargement. We are reporting in this paper the case of a 30-year-old Maldivian female who presented to our surgical OPD with a right cervical large solitary soft tissue lesion. All the investigations were negative except for a large highly vascularized unicentric soft tissue mass measuring around 4 cm in the anterior triangle of the neck seen by the ultrasound separable from all other surroundings. An excisional biopsy & complete resection was done under general anesthesia in the operation theater of a Maldivian Regional hospital. Unicentric Castleman's disease as a diagnosis was confirmed based on the final histopathological study of the specimen, and the absence of other cervical and extra-cervical lymphadenopathy. The patient doesn't have recurrence or newly developed lymph nodes at the time of reporting this article.
Neck lymph nodes are involved by CD and may be confused with other common causes of neck lymphadenopathy. The definitive diagnosis of CD is based on histopathological assessment and multiple treatment modalities have been tried. There are three types of CD: hyaline-vascular type (most common), plasma cell type and mixed variant type. The localized form of the disease is mostly asymptomatic with a single site lymph node enlargement. Surgical resection is considered the cornerstone of radical treatment for unicentric CD.
Collapse
|
|
24
|
Hua Y, Liang C, Yang J, Wang L, Xu A, Xi L, Wang S, Wang Z. Clinical characteristics and prognosis of patients with Castleman disease in a Chinese hospital: paraneoplastic pemphigus is an independent risk factor. Am J Transl Res 2022; 14:1051-1059. [PMID: 35273707 PMCID: PMC8902542] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2021] [Accepted: 12/20/2021] [Indexed: 06/14/2023]
Abstract
OBJECTIVE Castleman disease (CD) is a rare lymphoproliferative disorder with limited clinical research data. This study aimed to investigate the clinical manifestations, pathologic features, and prognostic factors of CD. METHODS The clinicopathological data of 54 patients with CD hospitalized in the First Affiliated Hospital of Nanjing Medical University were retrospectively analyzed. Univariate analysis and multivariate analysis were performed by Cox regression model to determine independent prognostic factors for patients' survival. RESULTS According to clinical classification, 30 cases (55.6%) had unicentric CD (UCD) and 24 cases (44.4%) had multicentric CD (MCD). Moreover, pathologic classification revealed 32 cases (59.3%) with hyaline vascular variant, 3 (5.6%) with mixed cellular variant, and 19 (35.2%) with plasmacytic variant. Patients with MCD commonly presented with clinical signs and symptoms, including fever, splenomegaly, and pleural effusion and/or ascites. Clinical complications, such as liver injury, anemia, and polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome were more common in patients with MCD. Univariate analysis showed that the presence of paraneoplastic pemphigus (PNP) and the elevation of C-reactive protein were unfavorable prognosticators of survival in patients with CD. By multivariate analysis PNP was an independent prognostic factor in patients with CD. CONCLUSIONS This study provided a panoramic elaboration of CD cases and showed that the presence of PNP was an independent unfavorable factor.
Collapse
Affiliation(s)
- Yibo Hua
- Department of Urology, The First Affiliated Hospital of Nanjing Medical UniversityNanjing 210029, Jiangsu Province, China
| | - Chao Liang
- Department of Urology, The First Affiliated Hospital of Nanjing Medical UniversityNanjing 210029, Jiangsu Province, China
| | - Jie Yang
- Department of Urology, The First Affiliated Hospital of Nanjing Medical UniversityNanjing 210029, Jiangsu Province, China
| | - Luyang Wang
- Department of Pathology, The First Affiliated Hospital of Nanjing Medical UniversityNanjing 210029, Jiangsu Province, China
| | - Aimin Xu
- Department of Urology, The First Affiliated Hospital of Nanjing Medical UniversityNanjing 210029, Jiangsu Province, China
| | - Lei Xi
- Department of Pathology, The First Affiliated Hospital of Nanjing Medical UniversityNanjing 210029, Jiangsu Province, China
| | - Shangqian Wang
- Department of Urology, The First Affiliated Hospital of Nanjing Medical UniversityNanjing 210029, Jiangsu Province, China
| | - Zengjun Wang
- Department of Urology, The First Affiliated Hospital of Nanjing Medical UniversityNanjing 210029, Jiangsu Province, China
| |
Collapse
|
|
25
|
Mohan M, Meek JC, Meek ME, Broadwater R, Alapat D, van Rhee F. Combinatorial treatment for unresectable unicentric Castleman disease. Eur J Haematol 2021; 107:484-488. [PMID: 34242421 DOI: 10.1111/ejh.13685] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/03/2021] [Revised: 07/06/2021] [Accepted: 07/06/2021] [Indexed: 01/20/2023]
Abstract
Unresectable, symptomatic unicentric Castleman disease (UCD) can represent a formidable therapeutic challenge. UCD masses are often highly vascularized offering the opportunity for therapeutic embolization. Herein, we report on 6 patients in which therapeutic embolization was combined with other medical interventions including surgery (n = 3), rituximab (n = 6), cryoablation (n = 2), and chemotherapy (n = 3). Five patients had significant tumor volume reductions (median: 83.2%; range: 76.7-100). All five responding patients had resolution of symptomatology. There were no serious complications in the patients who received embolization and proceeded to surgery. In conclusion, effective disease and symptom control can be obtained in patients with symptomatic, unresectable UCD by combining different therapeutic interventions.
Collapse
Affiliation(s)
- Meera Mohan
- Myeloma Center, University of Arkansas for Medical Sciences, Little Rock, AR, USA
| | - James C Meek
- Department of Radiology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
| | - Mary Elizabeth Meek
- Department of Radiology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
| | - Ralph Broadwater
- Department of Surgery, University of Arkansas for Medical Sciences, Little Rock, AR, USA
| | - Daisy Alapat
- Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
| | - Frits van Rhee
- Myeloma Center, University of Arkansas for Medical Sciences, Little Rock, AR, USA
| |
Collapse
|
|
26
|
International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. Blood Adv 2021; 4:6039-6050. [PMID: 33284946 DOI: 10.1182/bloodadvances.2020003334] [Citation(s) in RCA: 104] [Impact Index Per Article: 26.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/03/2020] [Accepted: 10/09/2020] [Indexed: 02/06/2023] Open
Abstract
Castleman disease (CD) includes a group of rare and heterogeneous disorders with characteristic lymph node histopathological abnormalities. CD can occur in a single lymph node station, which is referred to as unicentric CD (UCD). CD can also involve multicentric lymphadenopathy and inflammatory symptoms (multicentric CD [MCD]). MCD includes human herpesvirus-8 (HHV-8)-associated MCD, POEMS-associated MCD, and HHV-8-/idiopathic MCD (iMCD). The first-ever diagnostic and treatment guidelines were recently developed for iMCD by an international expert consortium convened by the Castleman Disease Collaborative Network (CDCN). The focus of this report is to establish similar guidelines for the management of UCD. To this purpose, an international working group of 42 experts from 10 countries was convened to establish consensus recommendations based on review of treatment in published cases of UCD, the CDCN ACCELERATE registry, and expert opinion. Complete surgical resection is often curative and is therefore the preferred first-line therapy, if possible. The management of unresectable UCD is more challenging. Existing evidence supports that asymptomatic unresectable UCD may be observed. The anti-interleukin-6 monoclonal antibody siltuximab should be considered for unresectable UCD patients with an inflammatory syndrome. Unresectable UCD that is symptomatic as a result of compression of vital neighboring structures may be rendered amenable to resection by medical therapy (eg, rituximab, steroids), radiotherapy, or embolization. Further research is needed in UCD patients with persisting constitutional symptoms despite complete excision and normal laboratory markers. We hope that these guidelines will improve outcomes in UCD and help treating physicians decide the best therapeutic approach for their patients.
Collapse
|
|
27
|
Guo Z, Liu C, Sun J, Zeng L, Zhang K. Castleman's disease of the left parotid gland: a case report. INTERNATIONAL JOURNAL OF CLINICAL AND EXPERIMENTAL PATHOLOGY 2021; 14:533-537. [PMID: 33936378 PMCID: PMC8085830] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 11/09/2020] [Accepted: 01/31/2021] [Indexed: 06/12/2023]
Abstract
Castleman's disease is a very rare heterogeneous group of lymphoproliferative disorders characterized by non-neoplastic growths. It is unknown about the pathophysiology of the Castleman's disease. Previous studies demonstrated that Castleman's disease can be divided into two groups according to clinical classification, including unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). The hyaline vascular type is most common in the head and neck, and is abbreviated as UCD. In the present case, a woman complained that a mass in her parotid gland was growing and it was painless seven months ago. The computed tomography (CT) showed that the superficial lobe of the parotid gland on the left had an elliptical soft tissue density shadow, about 2.5×3.5 cm, with clear boundaries and no obvious abnormalities in the surrounding bone. The CT scan showed no obvious abnormalities in the shape and density of the right parotid and bilateral submandibular glands. After the operation, combined with the results of immunohistochemistry, the final diagnosis was Castleman tumor. The patient recovered smoothly, after the operation and during follow-up. The patient maintained good health without recurrence or metastasis.
Collapse
Affiliation(s)
- Zhenfei Guo
- Department of Stomatology, The First Affiliated Hospital of Bengbu Medical College287 Chang Huai Road, Bengbu, Anhui, P. R. China
| | - Chang Liu
- Department of Stomatology, The First Affiliated Hospital of Bengbu Medical College287 Chang Huai Road, Bengbu, Anhui, P. R. China
| | - Jing Sun
- Department of Microbiology, Bengbu Medical College600 Dong Hai Avenue, Bengbu, Anhui, P. R. China
| | - Linggong Zeng
- Department of Pathology, The First Affiliated Hospital of Bengbu Medical College287 Chang Huai Road, Bengbu, Anhui, P. R. China
| | - Kai Zhang
- Department of Stomatology, The First Affiliated Hospital of Bengbu Medical College287 Chang Huai Road, Bengbu, Anhui, P. R. China
| |
Collapse
|
|
28
|
Wang W, Dong D, Wen J, Li H. A 10-year observational single-center study of retroperitoneal unicentric Castleman disease. Medicine (Baltimore) 2021; 100:e25088. [PMID: 33725899 PMCID: PMC7969295 DOI: 10.1097/md.0000000000025088] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/06/2020] [Accepted: 02/17/2021] [Indexed: 01/05/2023] Open
Abstract
Diagnosis of unicentric Castleman disease (UCD) is not easy before the resection and obtainment of pathological result. We retrospectively summarized 10-year experience of clinical evaluation and management for retroperitoneal UCD in Peking Union Medical College Hospital (PUMCH) between December 1, 2009 and December 31, 2019. Seventy two UCD patients with pathological diagnosis after resection were screened out. Among them 25 patients had retroperitoneal UCD. The average age of the 25 patients was 43.80 ± 12.79, and 52.00% were male. No patients had systemic symptoms, and 1 patient got preoperative treatment. The average size of masses was 5.59 ± 2.86 cm. The UCD sites included kidney, adrenal area, perinephric area, pancreas, peripancreatic area, area of descending part of duodenum, periaortic area or beside iliac artery, and others. The masses presented different degree of enhancement on CT scans and hypoecho or isoecho on ultrasound. Increased metabolism could be found on 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET/CT). Some patients had positive results on somatostatin receptor imaging, but none had positive results on 131I-metaiiodo-benzylguanidine (131I-MIBG). Some patients presented the elevated level of interleukin-6 (IL-6), 24hour-urinary catecholamine and tumor markers. All the patients received complete resection of masses and 96.00% had hyaline-vascular type pathology except 1 patient (plasma cell-type). Ninety two percent patients received a long-term follow-up with an average follow-up time of 35.48 ± 33.90 months. No patients died or experienced relapse during follow-up. Differential diagnosis of retroperitoneal UCD may be difficult according to imaging and laboratorial examinations. Differential diagnosis with pheochromocytomas/paragangliomas should be taken into special consideration. Different imaging examinations, such as CT/MRI, 18F-PET/CT, somatostatin receptor imaging and 131I-MIBG, can be combined for differential analysis. Complete resection is the best treatment and could provide a final pathological diagnosis.
Collapse
|
|
29
|
Chen H, Ba W, Chen H, Yang H. Cutaneous involvement as initial presentation of multicentric plasmacytic Castleman disease. J Cutan Pathol 2021; 48:701-705. [PMID: 33469928 DOI: 10.1111/cup.13964] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2020] [Revised: 01/09/2021] [Accepted: 01/12/2021] [Indexed: 01/09/2023]
Abstract
Castleman disease (CD) is an unusual lymphoproliferative disorder characterized by multiple lymphadenopathy accompanied by marked systemic inflammatory symptoms. CD can be unicentric (UCD) or multicentric (MCD), and it can be classified into three types based on histopathology: hyaline vascular type, plasma cell type, and mixed hyaline vascular and plasma cell type. CD involving skin is an unusual clinical manifestation. Abnormalities including rash, hyperpigmentation, cherry hemangiomatosis, paraneoplastic pemphigus, and Kaposi sarcoma have been reported to occur in MCD. Here, we reported an unusual case of MCD which presented initially with disseminated dark brown papules, patches, and plaques, and pathologically demonstrated plasma cell type CD, a finding which is rarely reported. The peculiar clinicopathological features will be discussed.
Collapse
Affiliation(s)
- Hongxiao Chen
- Department of Dermatology, Linyi People's Hospital, Shandong, China
| | - Wei Ba
- Department of Dermatology, Chinese PLA General Hospital and Medical School, Beijing, China
| | - Hao Chen
- Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, China
| | - Hongyu Yang
- Department of Pathology, St. Vincent Evansville Medical Center, Evansville, Indiana, USA
| |
Collapse
|
|
30
|
Kim IK, Lee KY. Adult Langerhans cell histiocytosis of skull in a patient with synchronous papillary thyroid carcinoma and Castleman disease. BMJ Case Rep 2021; 14:14/1/e239341. [PMID: 33495189 PMCID: PMC7839860 DOI: 10.1136/bcr-2020-239341] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
We report an extremely rare case of adult Langerhans cell histiocytosis (LCH) in a patient with papillary thyroid carcinoma (PTC) and Castleman disease (CD). A 46-year-old man visited our hospital with anaemia; systemic imaging showed an abdominal and a left thyroid mass. Biopsy confirmed CD for the abdominal mass and PTC for the thyroid mass. Two months after, he presented with headache and a right parietal lump. Brain CT and enhanced MRI revealed an osteolytic mass with enhancement in the right parietal skull. Surgical removal and biopsy confirmed the diagnosis of skull LCH. The BRAF mutation was positive on PTC and negative on CD and LCH. We conducted surgical resection only for PTC and LCH; surgical resection with siltuximab for multicentric CD. At the 25-month follow-up, there was no recurrence or progression. We may consider of syndromic nature of these diseases to establish a treatment strategy.
Collapse
Affiliation(s)
- In Kyeong Kim
- Neurosurgery, Kangwon National University Hospital, Chuncheon, Kangwondo, Korea (the Republic of)
| | - Kyoung Yul Lee
- Pathology, Kangwon National University Hospital, Chuncheon, Kangwondo, Korea (the Republic of)
| |
Collapse
|
|
31
|
Pierson SK, Khor JS, Ziglar J, Liu A, Floess K, NaPier E, Gorzewski AM, Tamakloe MA, Powers V, Akhter F, Haljasmaa E, Jayanthan R, Rubenstein A, Repasky M, Elenitoba-Johnson K, Ruth J, Jacobs B, Streetly M, Angenendt L, Patier JL, Ferrero S, Zinzani PL, Terriou L, Casper C, Jaffe E, Hoffmann C, Oksenhendler E, Fosså A, Srkalovic G, Chadburn A, Uldrick TS, Lim M, van Rhee F, Fajgenbaum DC. ACCELERATE: A Patient-Powered Natural History Study Design Enabling Clinical and Therapeutic Discoveries in a Rare Disorder. CELL REPORTS MEDICINE 2020; 1:100158. [PMID: 33377129 PMCID: PMC7762771 DOI: 10.1016/j.xcrm.2020.100158] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 07/24/2020] [Revised: 10/29/2020] [Accepted: 11/19/2020] [Indexed: 01/09/2023]
Abstract
Geographically dispersed patients, inconsistent treatment tracking, and limited infrastructure slow research for many orphan diseases. We assess the feasibility of a patient-powered study design to overcome these challenges for Castleman disease, a rare hematologic disorder. Here, we report initial results from the ACCELERATE natural history registry. ACCELERATE includes a traditional physician-reported arm and a patient-powered arm, which enables patients to directly contribute medical data and biospecimens. This study design enables successful enrollment, with the 5-year minimum enrollment goal being met in 2 years. A median of 683 clinical, laboratory, and imaging data elements are captured per patient in the patient-powered arm compared with 37 in the physician-reported arm. These data reveal subgrouping characteristics, identify off-label treatments, support treatment guidelines, and are used in 17 clinical and translational studies. This feasibility study demonstrates that the direct-to-patient design is effective for collecting natural history data and biospecimens, tracking therapies, and providing critical research infrastructure.
Collapse
Affiliation(s)
- Sheila K Pierson
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Johnson S Khor
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Jasira Ziglar
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Amy Liu
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Katherine Floess
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Erin NaPier
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Alexander M Gorzewski
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Mark-Avery Tamakloe
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Victoria Powers
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Faizaan Akhter
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Eric Haljasmaa
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Raj Jayanthan
- Castleman Disease Collaborative Network, Philadelphia, PA 19104, USA
| | - Arthur Rubenstein
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Mileva Repasky
- Castleman Disease Collaborative Network, Philadelphia, PA 19104, USA
| | - Kojo Elenitoba-Johnson
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Jason Ruth
- Castleman Disease Collaborative Network, Philadelphia, PA 19104, USA
| | - Bette Jacobs
- Castleman Disease Collaborative Network, Philadelphia, PA 19104, USA
| | - Matthew Streetly
- Myeloma and Plasma Cell Disorders, King's College London, London SE1 9RT, UK
| | - Linus Angenendt
- Department of Medicine A, University Hospital Münster, Münster 48149, Germany
| | - Jose Luis Patier
- Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal, Madrid 28034, Spain
| | - Simone Ferrero
- Dipartimento di Biotecnologie Molecolari e Scienze per la Salute, Università degli Studi di Torino, via Genova 3, Torino 10126, Italy
| | - Pier Luigi Zinzani
- IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seràgnoli," Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale Università degli Studi, Bologna 40138, Italy
| | - Louis Terriou
- Service de Médecine Interne, Institute for Translational Research in Inflammation University of Lille, Inserm, CHU Lille, 59000 Lille, France
| | - Corey Casper
- Infectious Disease Research Institute, Seattle, WA 98102, USA
| | - Elaine Jaffe
- National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA
| | - Christian Hoffmann
- ICH Study Center, Infektionsmedizinisches Centrum Hamburg, Hamburg 20095, Germany
| | - Eric Oksenhendler
- Department of Clinical Immunology, Hôpital Saint-Louis, 75010 Paris, France
| | - Alexander Fosså
- Department of Oncology, Oslo University Hospital, 0188 Oslo, Norway
| | - Gordan Srkalovic
- Clinical Trials Department, Sparrow Herbert-Herman Cancer Center, Lansing, MI 48912, USA
| | - Amy Chadburn
- Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY 10065, USA
| | - Thomas S Uldrick
- Vaccine and Infectious Diseases Division, Fred Hutchinson Cancer Research Center, Seattle, WA 98109, USA
| | - Megan Lim
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Frits van Rhee
- Myeloma Center, Winthrop P. Rockefeller Cancer Institute, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA
| | - David C Fajgenbaum
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| |
Collapse
|
|
32
|
Korbi AE, Jellali S, Jguiri M, Bellalah A, Ferjaoui M, Bouatay R, Kolsi N, Harrathi K, Koubaa J. Castelman's disease of the neck: a case report and literature review. Pan Afr Med J 2020; 37:369. [PMID: 33796182 PMCID: PMC7992414 DOI: 10.11604/pamj.2020.37.369.26909] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2020] [Accepted: 11/14/2020] [Indexed: 11/26/2022] Open
Abstract
Castleman’s disease is a rare pathology, poorly understood. It is considered as a lymphoproliferative disorder, described for the first time in 1954, which may be confused with other causes of lymphadenopathy. We report in this paper the case of a young women presenting with left latero-cervical lymphadenopathy. All the investigations were negative except a large high-vascularized level II cervical lymphadenopathy. We performed a cervicotomy. The extemporaneous histological exam was non-contributive. We decided to perform a complete level II and III left cervical lymphadenectomy. The diagnosis of unicentric Castleman's disease was confirmed based on the final histological study of the specimen, and the absence of other cervical and extra-cervical lymphadenopathy. The patient is free of recurrence at the time of reporting this article.
Collapse
Affiliation(s)
- Amel El Korbi
- ENT Department of Fattouma Bourguiba, University Hospital of Monastir, Monastir, Tunisia.,Research Unit "Quality and Security of care" (UR12SP41), University of Monastir, Monastir, Tunisia
| | - Sondes Jellali
- ENT Department of Fattouma Bourguiba, University Hospital of Monastir, Monastir, Tunisia
| | - Mahbouba Jguiri
- Department of Rheumatology, Fattouma Bourguiba University Hospital of Monastir, Monastir, Tunisia
| | - Ahlem Bellalah
- Anatomical and Cytological Pathology Department, Fattouma Bourguiba University Hospital of Monastir, Monastir, Tunisia
| | - Mehdi Ferjaoui
- ENT Department of Fattouma Bourguiba, University Hospital of Monastir, Monastir, Tunisia
| | - Rachida Bouatay
- ENT Department of Fattouma Bourguiba, University Hospital of Monastir, Monastir, Tunisia
| | - Naourez Kolsi
- ENT Department of Fattouma Bourguiba, University Hospital of Monastir, Monastir, Tunisia.,Research Unit "Quality and Security of care" (UR12SP41), University of Monastir, Monastir, Tunisia
| | - Khaled Harrathi
- ENT Department of Fattouma Bourguiba, University Hospital of Monastir, Monastir, Tunisia
| | - Jamel Koubaa
- ENT Department of Fattouma Bourguiba, University Hospital of Monastir, Monastir, Tunisia
| |
Collapse
|
|
33
|
Koga T, Takemori S, Hagimori N, Morimoto S, Sumiyoshi R, Shimizu T, Hosogaya N, Fukushima C, Yamamoto H, Kawakami A. An open-label continuation trial of sirolimus for tocilizumab-refractory idiopathic multicentric Castleman disease: Study protocol for an investigator-initiated, multicenter, open-label trial (SPIRIT compliant). Medicine (Baltimore) 2020; 99:e23291. [PMID: 33327255 PMCID: PMC7738043 DOI: 10.1097/md.0000000000023291] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
BACKGROUND Interleukin 6 (IL-6) inhibitors are the first-line treatment for idiopathic multicentric Castleman disease (iMCD); however, there is no established treatment for cases that are resistant to IL-6 inhibitors. Although sirolimus, a mammalian target of rapamycin inhibitor, has been suggested to be effective in patients with iMCD, the long-term safety and efficacy of sirolimus on individuals with IL-6 inhibitor-resistant iMCD have not been evaluated. METHODS/DESIGN In this investigator-initiated, multicenter, open-label trial, the long-term safety of sirolimus will be evaluated in patients participating in a placebo-controlled, randomized, double-blind, parallel-group trial on tocilizumab (TCZ)-resistant iMCD. The study will be conducted in 7 centers in Japan. This trial will be promptly started after the evaluation and examination for 16 weeks in the preceding study. The trial will be completed by the time the drug is approved for iMCD treatment in Japan. The primary endpoint is the incidence of adverse events. The secondary endpoints include the following: the levels of hemoglobin, albumin, and C-reactive protein; change in CHAP score; physician global assessment (100-mm visual analog scale); patient global assessment (100-mm visual analog scale); and lymph node changes in subjects with lymphadenopathy. DISCUSSION This clinical trial will provide evidence regarding the long-term safety of sirolimus as a potential novel therapeutic agent for patients with tocilizumab-resistant iMCD. TRIAL REGISTRATION NUMBER jRCT2051200050.
Collapse
Affiliation(s)
- Tomohiro Koga
- Division of Advanced Preventive Medical Sciences, Department of Immunology and Rheumatology
- Center for Bioinformatics and Molecular Medicine, Nagasaki University Graduate School of Biomedical Sciences
| | - Sachiko Takemori
- Nagasaki University Hospital, Clinical Research Center, Nagasaki
| | - Naoko Hagimori
- Nagasaki University Hospital, Clinical Research Center, Nagasaki
- Translational Research Center for Medical Innovation, Foundation for Biomedical Research and Innovation at Kobe, Kobe, Japan
| | - Shimpei Morimoto
- Nagasaki University Hospital, Clinical Research Center, Nagasaki
| | - Remi Sumiyoshi
- Division of Advanced Preventive Medical Sciences, Department of Immunology and Rheumatology
- Nagasaki University Hospital, Clinical Research Center, Nagasaki
| | - Toshimasa Shimizu
- Division of Advanced Preventive Medical Sciences, Department of Immunology and Rheumatology
- Nagasaki University Hospital, Clinical Research Center, Nagasaki
| | - Naoki Hosogaya
- Nagasaki University Hospital, Clinical Research Center, Nagasaki
| | - Chizu Fukushima
- Nagasaki University Hospital, Clinical Research Center, Nagasaki
| | - Hiroshi Yamamoto
- Nagasaki University Hospital, Clinical Research Center, Nagasaki
| | - Atsushi Kawakami
- Division of Advanced Preventive Medical Sciences, Department of Immunology and Rheumatology
| |
Collapse
|
|
34
|
Fang X, Sun Z, Xu-Monette ZY, Young KH. Predictive Model for Idiopathic Multicentric Castleman Disease Supporting Treatment Decisions. Oncologist 2020; 26:4-6. [PMID: 33215784 DOI: 10.1002/onco.13605] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/25/2020] [Accepted: 10/28/2020] [Indexed: 01/04/2023] Open
Affiliation(s)
- Xiaosheng Fang
- Department of Pathology, Hematopathology Division, Duke University Medical Center, Durham, North Carolina, USA.,Department of Hematology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, People's Republic of China
| | - Zhongling Sun
- Department of Neurology, The People's Hospital of Zhaoyuan City, Shandong, People's Republic of China
| | - Zijun Y Xu-Monette
- Department of Pathology, Hematopathology Division, Duke University Medical Center, Durham, North Carolina, USA.,Duke Cancer Institute, Durham, North Carolina, USA
| | - Ken H Young
- Department of Pathology, Hematopathology Division, Duke University Medical Center, Durham, North Carolina, USA.,Duke Cancer Institute, Durham, North Carolina, USA
| |
Collapse
|
|
35
|
Han EJ, O JH, Jung SE, Park G, Choi BO, Jeon YW, Min GJ, Cho SG. FDG PET/CT Findings of Castleman Disease Assessed by Histologic Subtypes and Compared with Laboratory Findings. Diagnostics (Basel) 2020; 10:diagnostics10120998. [PMID: 33255420 PMCID: PMC7761046 DOI: 10.3390/diagnostics10120998] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2020] [Revised: 11/20/2020] [Accepted: 11/21/2020] [Indexed: 01/09/2023] Open
Abstract
Castleman disease (CD) is a relatively rare lymphoproliferative disorder and the pathophysiology of the subtypes are incompletely understood. Fluorine-18 fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) demonstrates the metabolic activity of inflammatory and tumorous conditions. The FDG uptake intensity and sites of involved lesions on FDG PET/CT were assessed by histologic subtypes, and compared to the patient's hemoglobin, platelet, albumin, and high-sensitivity C-reactive protein (hs-CRP) levels. In total, 60 PET/CT images of 44 consecutive CD patients were included: 4 (9%) unicentric and 40 (91%) multicentric; 21 (48%) hyaline vascular subtype, 16 (36%) plasma cell, and 7 (16%) mixed or unclassified. The maximum standardized uptake value (SUVmax) and tumor-to-liver (T/L) ratio of involved lymph nodes (LNs) were 5.3 ± 2.4 (range, 1.6-11.5) and 2.8 ± 1.6 (range, 1.1-9.6), respectively, with no significant difference between the histologic subtypes. Higher number of involved LN stations and presence of extra-nodal involvement on FDG PET/CT were associated with thrombocytopenia, hypoalbuminemia, and elevated hs-CRP levels (p values < 0.05). FDG-avidity was not different by histologic subtypes and did not correlate with laboratory findings. However, the extent of nodal and extra-nodal involvement as noted on FDG PET/CT was significantly associated with abnormal laboratory findings in patients with CD.
Collapse
Affiliation(s)
- Eun Ji Han
- Division of Nuclear Medicine, Department of Radiology, Yeouido St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea;
| | - Joo Hyun O
- Division of Nuclear Medicine, Department of Radiology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea
- Correspondence:
| | - Seung-Eun Jung
- Department of Radiology, Eunpyeong St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea;
| | - Gyeongsin Park
- Department of Hospital Pathology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea;
| | - Byung-Ock Choi
- Department of Radiation Oncology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea;
| | - Young-Woo Jeon
- Department of Hematology, Yeouido St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea;
| | - Gi-June Min
- Department of Hematology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea; (G.-J.M.); (S.-G.C.)
| | - Seok-Goo Cho
- Department of Hematology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea; (G.-J.M.); (S.-G.C.)
| |
Collapse
|
|
36
|
Unicentric Castleman's Disease Masquerading as Retroperitoneal Sarcoma-Two Paraneoplastic Presentations of a Rare Disease. Indian J Surg Oncol 2020; 11:78-83. [PMID: 33088135 DOI: 10.1007/s13193-020-01072-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2019] [Accepted: 04/01/2020] [Indexed: 10/24/2022] Open
|
|
37
|
Yu L, Shi M, Cai Q, Strati P, Hagemeister F, Zhai Q, Li L, Fang X, Li J, Sun R, Zhang S, Yang H, Wang Z, Qian W, Iwaki N, Sato Y, Zhang L, Li J, Oksenhendler E, Xu-Monette ZY, Young KH. A Novel Predictive Model for Idiopathic Multicentric Castleman Disease: The International Castleman Disease Consortium Study. Oncologist 2020; 25:963-973. [PMID: 32852137 DOI: 10.1634/theoncologist.2019-0986] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/23/2019] [Accepted: 07/21/2020] [Indexed: 12/25/2022] Open
Abstract
BACKGROUND Patients with multicentric Castleman disease (MCD) who are negative for human immunodeficiency virus and human herpesvirus 8 are considered to have idiopathic MCD (iMCD). The clinical presentation of iMCD varies from mild constitutional symptoms to life-threatening symptoms or death. The treatment strategy varies from "watchful waiting" to high-dose chemotherapy. This diverse clinical presentation calls for a classification stratification system that takes into account the severity of the disease. SUBJECTS, MATERIALS, AND METHODS We analyzed the clinical, laboratory, and pathologic abnormalities and treatment outcomes of 176 patients with iMCD (median follow-up duration 12 years) from the U.S. and China to better understand the characteristics and prognostic factors of this disease. This discovery set of iMCD results was confirmed from the validation set composed of additional 197 patients with iMCD organized from The International Castleman Disease Consortium. RESULTS Using these data, we proposed and validated the iMCD international prognostic index (iMCD-IPI), which includes parameters related to patient characteristics (age > 40 years), histopathologic features (plasma cell variant), and inflammatory consequences of iMCD (hepatomegaly and/or splenomegaly, hemoglobin <80 g/L, and pleural effusion). These five factors stratified patients according to their performance status and extent of organ dysfunction into three broad categories: low risk, intermediate risk, and high risk. The iMCD-IPI score accurately predicted outcomes in the discovery study cohort, and the results were confirmed on the validation study cohort. CONCLUSION This study represents the largest series of studies on patients with iMCD in the field and proposed a novel risk-stratification model for iMCD-IPI that could be used to guide risk-stratified treatment strategies in patients with iMCD. IMPLICATIONS FOR PRACTICE Patients with idiopathic multicentric Castleman disease (iMCD) can benefit from care based on clinical symptoms and disease severity. This study in 176 patients with iMCD constructed an iMCD-IPI score based on five clinical factors, including age >40 years, plasmacytic variant subtype, hepatomegaly and/or splenomegaly, hemoglobin <80 g/L, and pleural effusion, and stratified patients into three risk categories: low risk, intermediate risk, and high risk. The predictive value was validated in an independent set of 197 patients with iMCD from The International Castleman Disease Consortium. The proposed novel model is valuable for predicting clinical outcome and selecting optimal therapies using clinical parameters.
Collapse
Affiliation(s)
- Li Yu
- Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang University, NanChang, People's Republic of China
- Division of Hematopathology and Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA
| | - Menghan Shi
- Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang University, NanChang, People's Republic of China
| | - Qingqing Cai
- State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Sun Yat-sen University Cancer Center, Guangzhou, People's Republic of China
| | - Paolo Strati
- Departments of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
| | - Fredrick Hagemeister
- Departments of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
| | - Qiongli Zhai
- Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, Tianjin, People's Republic of China
| | - Ling Li
- Department of Medical Oncology, Zhengzhou University Cancer Center, Zhengzhou, People's Republic of China
| | - Xiaosheng Fang
- Department of Hematology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Ji'nan, Shandong, People's Republic of China
| | - Jianyong Li
- Department of Hematology and Oncology, Jiangsu Province Hospital, Nanjing University, Nanjing, People's Republic of China
| | - Ruifang Sun
- Department of Pathology, Shanxi Cancer Hospital, Taiyuan, Shanxi, People's Republic of China
| | - Shanxiang Zhang
- Department of Pathology, Indiana University School of Medicine, Indianapolis, Indiana, USA
| | - Hanjin Yang
- Department of Pathology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China
| | - Zhaoming Wang
- Department of Pathology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China
| | - Wenbin Qian
- Department of Hematology and Oncology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China
| | - Noriko Iwaki
- Department of Hematology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan
| | - Yasuharu Sato
- Division of Pathophysiology, Okayama University Graduate School of Health Sciences, Okayama, Japan
| | - Lu Zhang
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China
| | - Jian Li
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China
| | - Eric Oksenhendler
- Département d'Immunologie Clinique, Hôpital Saint-Louis, Paris, France
| | - Zijun Y Xu-Monette
- Division of Hematopathology and Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA
| | - Ken H Young
- Division of Hematopathology and Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA
- Duke Cancer Institute, Durham, North Carolina, USA
| |
Collapse
|
|
38
|
Wang J, Liang LJ, Wang YM, Mei ZY, Liu YZ, Liu LN, Fang BJ, Song YP. [Analysis of the clinicopathologic features as well as diagnosis and treatment of 59 patients with Castleman disease]. ZHONGHUA XUE YE XUE ZA ZHI = ZHONGHUA XUEYEXUE ZAZHI 2020; 41:666-670. [PMID: 32942821 PMCID: PMC7525172 DOI: 10.3760/cma.j.issn.0253-2727.2020.08.009] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/10/2020] [Indexed: 12/12/2022]
Abstract
Objective: To investigate the clinicopathologic features, treatment, and prognosis in patients with Castleman disease (CD) . Methods: We retrospectively analyzed the clinicopathologic data of 59 patients for whom a diagnosis of Castleman disease was confirmed using pathological examination from October 2011 to October 2019 at the Henan Cancer Hospital. The patients were divided into the following two groups as per the following clinical classifications: unicentric CD (UCD, n=47) and multicentric CD (MCD, n=12) . Data on clinical manifestations, laboratory findings, treatment, and prognosis were analyzed. Results: There was no significant difference in the median age and the ratio of male to female between the UCD and MCD. UCD was characterized by asymptomatic enlargement of the single lymph node. The main pathological type was hyaline vascular histopathology (83.0%) . Of these, 44 patients chose surgical resection, and their prognosis was good. Treatment. MCD was characterized by multiple enlarged superficial and/or deep lymph nodes with B symptoms, weakness, and hepatosplenomegaly. Anemia, hypoproteinemia, and globulin level were increased on laboratory examinations. Plasmacyte histopathology was the main pathological type and was present in about 50.0% of the subjects. Only chemotherapy was performed for these MCD patients, followed by chemotherapy or chemotherapy followed by radiotherapy, and the efficient was 58.3% (7/12) . Conclusions: UCD, characterized by asymptomatic enlargement of the single lymph node, shows good postoperative prognosis. MCD has relatively complex clinical manifestations and poor prognosis, and optimal treatment is yet to be established.
Collapse
Affiliation(s)
- J Wang
- Department of Hematology, the Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou 450008, China
| | - L J Liang
- Department of Hematology, the Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou 450008, China
| | - Y M Wang
- Department of Hematology, the Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou 450008, China
| | - Z Y Mei
- Department of Hematology, the Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou 450008, China
| | - Y Z Liu
- Department of Hematology, the Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou 450008, China
| | - L N Liu
- Department of Hematology, the Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou 450008, China
| | - B J Fang
- Department of Hematology, the Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou 450008, China
| | - Y P Song
- Department of Hematology, the Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou 450008, China
| |
Collapse
|
|
39
|
Wang L, Nong L, Li F, Wang X, Wang R, Chen X, Tu P, Dong Y, Li T, Zhu X, Wang M. Predominant Stroma-Rich Feature in Hyaline Vascular Variant of Castleman Disease Is Associated With Paraneoplastic Pemphigus. Am J Clin Pathol 2020; 154:403-413. [PMID: 32459333 DOI: 10.1093/ajcp/aqaa053] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022] Open
Abstract
OBJECTIVES We aimed to describe the clinical and histopathologic features of Castleman disease (CD), particularly emphasizing its associations with paraneoplastic pemphigus (PNP) and prognosis. METHODS We retrospectively enrolled 123 CD patients at our center. Clinical, pathologic, and laboratory data were reviewed. RESULTS Fifty percent of the patients had PNP. Compared with those without PNP, patients with PNP-associated CD had more hyaline vascular (HV) variants (83.9% vs 57.4%), fewer mixed cellular variants (16.1% vs 24.6%), and no plasmacytic variants (0% vs 18.0%). Thirty-eight of 87 patients with the HV variant of CD (HV-CD) had stroma-rich (SR) features, and the incidence rate was higher in those with PNP-associated CD than in those without PNP (48.4% vs 13.1%, P < .001). The SR variant was associated with higher PNP-associated IgG titers than SR absence before surgery (median 1:160 vs 1:80, P = .019) or after surgery (median 1:160 vs 1:40, P = .013). The SR variant was also an unfavorable prognostic factor for CD survival in univariate analysis. The 3-year survival rates were 47.5% among those with PNP and 87.7% among those without PNP (P < .001). CONCLUSIONS PNP is associated with specific subtypes of CD and affects survival. The SR variant of HV-CD positively correlates with the incidence of PNP.
Collapse
Affiliation(s)
- Leyi Wang
- Department of Dermatology, Peking University First Hospital, Beijing, China
- National Clinical Research Center for Skin and Immune Diseases, Beijing, China
- Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China
| | - Lin Nong
- Department of Pathology, Peking University First Hospital, Beijing, China
| | - Furong Li
- Department of Dermatology, Peking University First Hospital, Beijing, China
- National Clinical Research Center for Skin and Immune Diseases, Beijing, China
- Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China
| | - Xue Wang
- Department of Dermatology, Peking University First Hospital, Beijing, China
- National Clinical Research Center for Skin and Immune Diseases, Beijing, China
- Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China
| | - Rui Wang
- Department of Dermatology, Peking University First Hospital, Beijing, China
- National Clinical Research Center for Skin and Immune Diseases, Beijing, China
- Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China
| | - Xixue Chen
- Department of Dermatology, Peking University First Hospital, Beijing, China
- National Clinical Research Center for Skin and Immune Diseases, Beijing, China
- Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China
| | - Ping Tu
- Department of Dermatology, Peking University First Hospital, Beijing, China
- National Clinical Research Center for Skin and Immune Diseases, Beijing, China
- Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China
| | - Yujun Dong
- Department of Hematology, Peking University First Hospital, Beijing, China
| | - Ting Li
- Department of Pathology, Peking University First Hospital, Beijing, China
| | - Xuejun Zhu
- Department of Dermatology, Peking University First Hospital, Beijing, China
- National Clinical Research Center for Skin and Immune Diseases, Beijing, China
- Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China
| | - Mingyue Wang
- Department of Dermatology, Peking University First Hospital, Beijing, China
- National Clinical Research Center for Skin and Immune Diseases, Beijing, China
- Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China
| |
Collapse
|
|
40
|
Koga T, Hagimori N, Takemori S, Morimoto S, Sumiyoshi R, Shimizu T, Hosogaya N, Fukushima C, Yamamoto H, Kawakami A. Randomized, double-blind, placebo-controlled, parallel-group trial of sirolimus for tocilizumab-resistant idiopathic multicentric Castleman disease: Study protocol for clinical trial. Medicine (Baltimore) 2020; 99:e20710. [PMID: 32791665 PMCID: PMC7387047 DOI: 10.1097/md.0000000000020710] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022] Open
Abstract
BACKGROUND Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder of unknown etiology with systemic symptoms that include fever, night sweats, weight loss, and fatigue. Although tocilizumab (TCZ), which is a recombinant, humanized, anti-human interleukin 6 receptor monoclonal antibody, has been recommended to treat patients with iMCD, 40% of patients with iMCD do not achieve complete remission with TCZ treatment. METHODS/DESIGN In this phase II, investigator-initiated, multicenter, double-blind, randomized, parallel-group trial, the efficacy and safety of sirolimus will be compared with placebo in patients with TCZ-resistant iMCD. The study will be conducted in 8 centers in Japan. Participants (n = 20) will be randomly assigned to receive 2 mg of oral sirolimus (n = 10) or placebo (n = 10) once daily for 16 weeks. The primary endpoint is a decrease in CHAP score by ≥1 from baseline at 16 weeks. Secondary endpoints include levels of hemoglobin, albumin, and C-reactive protein; change in CHAP score; SF-36 Health Survey Questionnaire; physician global assessment (100 mm visual analog scale); patient global assessment (100 mm visual analog scale) at 2, 4, 8, 12, and 16 weeks; change in lymphadenopathy at 16 weeks; and pharmacodynamic assessment, including the measurement of whole blood sirolimus level. DISCUSSION This clinical trial will provide evidence of efficacy and safety of sirolimus as a potential new therapeutic agent for patients with TCZ-resistant iMCD. TRIAL REGISTRATION This study was registered with the Japan Registry of Clinical Trials as jRCT2071190029 on October 8, 2019.
Collapse
Affiliation(s)
- Tomohiro Koga
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences
- Center for Bioinformatics and Molecular Medicine, Nagasaki University Graduate School of Biomedical Sciences
| | - Naoko Hagimori
- Nagasaki University Hospital, Clinical Research Center, Nagasaki, Japan
| | - Sachiko Takemori
- Nagasaki University Hospital, Clinical Research Center, Nagasaki, Japan
| | - Shimpei Morimoto
- Nagasaki University Hospital, Clinical Research Center, Nagasaki, Japan
| | - Remi Sumiyoshi
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences
- Nagasaki University Hospital, Clinical Research Center, Nagasaki, Japan
| | - Toshimasa Shimizu
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences
- Nagasaki University Hospital, Clinical Research Center, Nagasaki, Japan
| | - Naoki Hosogaya
- Nagasaki University Hospital, Clinical Research Center, Nagasaki, Japan
| | - Chizu Fukushima
- Nagasaki University Hospital, Clinical Research Center, Nagasaki, Japan
| | - Hiroshi Yamamoto
- Nagasaki University Hospital, Clinical Research Center, Nagasaki, Japan
| | - Atsushi Kawakami
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences
| |
Collapse
|
|
41
|
A lymphoproliferative pericardial mass. Proc (Bayl Univ Med Cent) 2020; 33:660-661. [PMID: 33116350 DOI: 10.1080/08998280.2020.1792750] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022] Open
Abstract
Castleman disease (CD) is a rare lymphoproliferative disorder with variable presentation and prognosis. Most CD cases are unicentric and correspond to the hyaline-vascular variant, a histopathological classification associated with better outcomes, which commonly presents as an enhancing hypervascular mediastinal mass. CD is often asymptomatic and surgically resectable. Nonetheless, surgical resection can be difficult when the lymphoid mass is causing compression of vital structures. We discuss a rare case of hyaline-vascular unicentric CD presenting as an incidental pericardial mass.
Collapse
|
|
42
|
Chisholm KM, Fleming MD. Histologic and Laboratory Characteristics of Symptomatic and Asymptomatic Castleman Disease in the Pediatric Population. Am J Clin Pathol 2020; 153:821-832. [PMID: 32112075 DOI: 10.1093/ajcp/aqaa011] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022] Open
Abstract
OBJECTIVES Compare the morphologic, laboratory, and clinical features of asymptomatic and symptomatic Castleman disease in the pediatric population. METHODS We reviewed clinical records and histopathology of patients with Castleman disease from 2 pediatric institutions. RESULTS Of 39 patients with pediatric Castleman disease, 37 had unicentric disease, all classified with the hyaline vascular variant of Castleman disease, 8 of which were clinically symptomatic. These 8 patients demonstrated abnormal laboratory findings, including microcytic anemia, elevated erythrocyte sedimentation rate and C-reactive protein, and hypoalbuminemia. In addition, histopathologic evaluation showed that the 8 symptomatic cases had more hyperplastic germinal centers, fewer atrophic or regressed germinal centers, fewer mantle zones containing multiple germinal centers, reduced "onion skinning" of mantle zones, and fewer "lollipop" formations compared with the asymptomatic cases. CONCLUSIONS This series of pediatric Castleman disease showed that lymph nodes from asymptomatic patients generally demonstrated the more classic hyaline vascular histology, whereas those with symptoms could lack or have only focal classic findings. As such, reactive lymph nodes with subtle Castleman-like features should prompt clinical correlation to ensure proper diagnosis.
Collapse
Affiliation(s)
- Karen M Chisholm
- Department of Laboratories, Seattle Children’s Hospital, Seattle, WA
- Department of Laboratory Medicine, University of Washington, Seattle
| | - Mark D Fleming
- Department of Pathology, Boston Children’s Hospital, Boston, MA
| |
Collapse
|
|
43
|
Zhang XX, Jiang L, Wang CY, Gu YY, Li LG, Xia TT, Huang Y, Huang PK, Zhang QL. [Clinical analysis of 30 cases of Castleman disease with different types of thoracic involvement]. ZHONGHUA XUE YE XUE ZA ZHI = ZHONGHUA XUEYEXUE ZAZHI 2020; 41:149-156. [PMID: 32135633 PMCID: PMC7357939 DOI: 10.3760/cma.j.issn.0253-2727.2020.02.012] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/19/2019] [Indexed: 11/18/2022]
Abstract
Objective: To improve the clinical understanding of Castleman disease (CD) with different types of thoracic involvement, including their clinical features, radiological and pathological findings, diagnosis and current treatment strategies. Methods: Retrospective analysis of 30 patients diagnosed with CD with thoracic involvement and hospitalized between June 2009 and May 2019 in The First Affiliated Hospital of Guangzhou Medical University was performed. Patients were divided into three groups for subsequent analysis based on the clinical data: CD with bronchiolitis obliterans (BO) , unicentric Castleman disease (UCD) without BO, and multicentric Castleman disease (MCD) without BO. Results: Among the 30 patients, there were 5 (16.7%) patients diagnosed with BO, 18 (60.0%) patients had UCD without BO and 7 (23.3%) patients had MCD without BO. The average age of MCD without BO patients was significantly older than that of BO and UCD without BO patients[ (49.29±5.39) ys vs (27.20±3.76) ys and (37.17±2.87) ys; P=0.005 and 0.034, respectively) ]. Pulmonary symptoms were commonly seen in BO group (100%) and MCD without BO group (71.4%) . while no pulmonary symptoms were seen in UCD without BO group. Key abnormal laboratory findings were erythrocyte sedimentation rate (ESR) increase (40%in BO group and 57.1% in MCD without BO group) and hypoxia (60% in BO group and 28.6% in MCD without BO group) . Other abnormal laboratory findings seen in MCD without BO group included anemia and IgG increase (both 57.1%) . Notably, all patients in BO group had extremely severe mixed ventilation dysfunction in the lung function test. CT scan showed lung parenchyma involvement in BO group (100%) , in UCD without BO group (11.1%) featured by solitary pulmonary nodule and in MCD without BO group (57.1%) featured by diffuse lesions in bilateral lungs. The size of lymph nodes was significantly smaller in MCD without BO group comparing to that in BO group and UCD without BO group[short diameter (1.83±0.51) cm vs (4.73±1.63) cm and (3.62±0.26) cm; P=0.006 and 0.011, respectively]. All patients (100%) in the BO group had a pathological type of transparent vascular variant while the same pathological type accounts for 88.9% in UCD without BO patients. The predominantly pathological type (57.1%) was plasma cell variant in the MCD without BO group. Oral ulcers presented in all patients in BO group but were relieved after the mass resection and immunomodulatory therapy, but the pulmonary symptoms were still progressively aggravated. Thoracoscopic mass excision was the main treatment for UCD without BO patients while chemotherapy, immunomodulatory and targeted therapy were commonly used for MCD without BO treatment. Conclusion: The age, clinical symptom, laboratory finding, lung function, imaging manifestation, pathology, treatment and prognosis were different among the three groups. This classification could improve clinical understanding of the disease.
Collapse
Affiliation(s)
- X X Zhang
- National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou Institute of Respiratory Health, Guangzhou 510120, China
| | - L Jiang
- Department of Respiratory and Critical Care Medicine, Huhhot First Hospital, Huhhot 010010, China
| | - C Y Wang
- Department of Hematology, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou 510120, China
| | - Y Y Gu
- National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou Institute of Respiratory Health, Guangzhou 510120, China
| | - L G Li
- National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou Institute of Respiratory Health, Guangzhou 510120, China
| | - T T Xia
- Department of Radiology, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou 510120, China
| | - Y Huang
- National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou Institute of Respiratory Health, Guangzhou 510120, China
| | - P K Huang
- Department of Respiratory Medicine, Huizhou Municipal Central Hospital, Huizhou 516001, China
| | - Q L Zhang
- National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou Institute of Respiratory Health, Guangzhou 510120, China
| |
Collapse
|
|
44
|
Liedtke KR, Waldburger N, Glitsch AS, Schreiber A. Castleman's disease mimicked melanoma metastasis in the mesentery - A case report. Int J Surg Case Rep 2020; 67:110-113. [PMID: 32058307 PMCID: PMC7016036 DOI: 10.1016/j.ijscr.2020.02.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2019] [Revised: 01/20/2020] [Accepted: 02/01/2020] [Indexed: 11/30/2022] Open
Abstract
Castleman’s disease (CD) is a rare finding and diagnosis is very challenging. Intraabdominal localization is uncommon and can pretend malignancy. Misinterpretation might lead to surgical overtreatment of asymptomatic patients. Close follow-up examination might help to distinguish between malignancy and CD. Introduction Castleman’s disease (CD) is a rare and mainly asymptomatic cause of lymph node swelling. Often it is unicentric and located in the mediastinum. Due to rarity of the disease as well as a lack of symptoms, diagnosis proves to be challenging, especially when CD affects another region. Presentation of case A 51-year old male underwent resection of a malignant melanoma. Further staging revealed an unclear abdominal mass located in the mesentery with close contact to small intestine. Under the assumption of metastasis, complete tumor removal including intestine resection and anastomosis was performed. Both, operation and postoperative phase proved uncomplicated. Surprisingly, however, histology revealed a benign lymphoproliferative disorder, CD. Discussion There are several differential diagnoses for abdominal soft tissue tumor, such as: gastrointestinal stromal tumor, sarcoma, lymphoma, or metastasis. In reference to the resected melanoma described above, metastasis was assumed with subsequent oncological resection. Both, the reliable detection of CD as well as the exclusion of malignant disease (e.g. lymphoma) can only be achieved through pathology, in that specific tests fail yet to exist. The etiology of CD remains barely understood and based upon few cases reported complete surgical resection is recommended. However, the common form is meant to be benign. Conclusion The potential diagnosis of CD should be made more common to surgeons, especially in completely asymptomatic patients and non-superficial lesions, whereby close follow-up examination might be offered to patients.
Collapse
Affiliation(s)
- K R Liedtke
- Department of General, Visceral, Thoracic and Vascular Surgery, University of Greifswald, Sauerbruchstraße, 17475 Greifswald, Germany.
| | - N Waldburger
- Institute of Pathology, University of Greifswald, Friedrich-Loeffler-Straße 23e, 17475 Greifswald, Germany
| | - A S Glitsch
- Department of General, Visceral, Thoracic and Vascular Surgery, University of Greifswald, Sauerbruchstraße, 17475 Greifswald, Germany
| | - A Schreiber
- Department of General, Visceral, Thoracic and Vascular Surgery, University of Greifswald, Sauerbruchstraße, 17475 Greifswald, Germany
| |
Collapse
|
|
45
|
Murakami M, Johkoh T, Hayashi S, Ohshima S, Mizuki M, Nakatsuka SI, Tomobe M, Kuroyanagi K, Nakasone A, Nishimoto N. Clinicopathologic characteristics of 342 patients with multicentric Castleman disease in Japan. Mod Rheumatol 2020; 30:843-851. [DOI: 10.1080/14397595.2019.1704983] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/09/2023]
Affiliation(s)
- Miho Murakami
- Osaka Rheumatology Clinic, Osaka, Japan
- Department of Molecular Regulation for Intractable Diseases, Institute of Medical Science, Tokyo Medical University, Tokyo, Japan
| | - Takeshi Johkoh
- Department of Radiology, Kansai Rosai Hospital, Hyogo, Japan
| | - Seiji Hayashi
- National Hospital Organization Kinki Chuo Chest Medical Center, Osaka, Japan
| | - Shiro Ohshima
- Department of Rheumatology and Allergology, National Hospital Organization, Osaka Minami Medical Center, Osaka, Japan
| | - Masao Mizuki
- Department of Chemotherapy, Osaka University Hospital, Osaka, Japan
| | - Shin-ichi Nakatsuka
- Department of Diagnostic Pathology and Cytology, Osaka International Cancer Institute, Osaka, Japan
| | - Minako Tomobe
- Chugai Pharmaceutical Co., Ltd., Tokyo, Japan; and Safety Evaluation Committee of tocilizumab for Castleman disease
| | - Kazuyuki Kuroyanagi
- Chugai Pharmaceutical Co., Ltd., Tokyo, Japan; and Safety Evaluation Committee of tocilizumab for Castleman disease
| | - Ayako Nakasone
- Chugai Pharmaceutical Co., Ltd., Tokyo, Japan; and Safety Evaluation Committee of tocilizumab for Castleman disease
| | - Norihiro Nishimoto
- Osaka Rheumatology Clinic, Osaka, Japan
- Department of Molecular Regulation for Intractable Diseases, Institute of Medical Science, Tokyo Medical University, Tokyo, Japan
| |
Collapse
|
|
46
|
Masaki Y, Kawabata H, Fujimoto S, Kawano M, Iwaki N, Kotani T, Nakashima A, Kurose N, Takai K, Suzuki R, Aoki S. Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan. J Clin Exp Hematop 2019; 59:175-178. [PMID: 31708515 PMCID: PMC6954167 DOI: 10.3960/jslrt.19021] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022] Open
Abstract
Castleman disease is a polyclonal lymphoproliferative disease which is clinically classified into unicentric (UCD) and multicentric (MCD). TAFRO syndrome is a relatively new concept that partly overlaps with MCD. Due to their rarity, their incidence remains unknown. This study investigated the incidence and prevalence of UCD, MCD, and TAFRO syndrome in Japan using a fixed-point observation method based on their incidence in Ishikawa prefecture. The annual incidences of MCD, UCD, and TAFRO syndrome in Japan were 309-731, 71-542, and 110-502, respectively, yielding annual incidence rates per million individuals of 2.4-5.8, 0.6-4.3, and 0.9-4.9, respectively, and nationwide prevalence of 4,180-14,900, 1,350-10,300, and 860-7,240, respectively. In conclusion, MCD, UCD and TAFRO syndrome may not be as rare as previously estimated in Japan.
Collapse
|
|
47
|
Clinicopathological Profile of Castleman's Disease in Indian Population: Experience From a Tertiary Care Center. Indian J Hematol Blood Transfus 2019; 36:254-259. [PMID: 32425374 DOI: 10.1007/s12288-019-01191-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2019] [Accepted: 09/13/2019] [Indexed: 10/25/2022] Open
Abstract
Castleman's disease (CD), also known as angiofollicular lymph node hyperplasia, a rare, non-malignant chronic lymphoproliferative disease characterized by, uni or multicentric lymphadenopathy. There is limited information about the clinicopathological variations and associations of this entity. A total of 50 cases of CD were retrieved from the archives, between the years 2005-2017. The cases were divided into pediatric (0-18 years), young adult (age ≤ 40 years) and older adult groups (age > 40 years respectively). Detailed clinicopathological correlation was done. The age range was 6-74 years. There was a male predominance (M: F-1.6:1). The majority (72%; 36/50) of the patients were adults; 46% young adult and 28% older adult, while only 28% (14/50) were of pediatric. Majority (78%) showed features of unicentric Castleman disease (UCD) while rest 22% presented with multi centric Castleman disease (MCD). Systemic symptoms were more frequent in MCD as compared to UCD cases (p = 0.06). The majority of the cases (40/50; 80%) were of the hyaline vascular type. Two of the cases showed mixed histological feature. Out of 50 patients 29 patient's treatment details are available. Majority unicentric cases were cases surgically excised 14/17 (82.3%). Three patients 3/17 (17.6%) were treated with chemotherapy. In multicentric Castleman's disease group, six of the 12 cases with MCD were managed by chemotherapy. One patient died of progressive disease. Castleman disease has varied clinical presentation and is often associated with other diseases. A high degree of suspicion and careful histological examination is required in order not to miss this entity.
Collapse
|
|
48
|
Rusconi A, Dario A, Agresta G, Balbi S. Acute Hydrocephalus Caused by Cerebellar Abscess in HIV-Negative Castleman's Disease. Asian J Neurosurg 2019; 14:1037-1039. [PMID: 31497160 PMCID: PMC6703033 DOI: 10.4103/ajns.ajns_32_17] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
Castleman's disease (CD) is a rare and indolent hematologic disorder characterized by solitary bulky adenopathy. Multiple nodal nonbulky localizations are described, while central nervous system involvement is rare. Immunodeficiency is associated with higher incidence of CD, and HIV serology should be performed at initial diagnosis. We report the case of a 64-year-old HIV-negative man affected by CD, presenting with acute hydrocephalus caused by a large cerebellar abscess.
Collapse
Affiliation(s)
- Angelo Rusconi
- Department of Biotechnology and Life Sciences, University of Insubria, Varese, Italy
| | | | - Gianluca Agresta
- Department of Biotechnology and Life Sciences, University of Insubria, Varese, Italy
| | - Sergio Balbi
- Department of Biotechnology and Life Sciences, University of Insubria, Varese, Italy
| |
Collapse
|
|
49
|
González-García A, Patier de la Peña JL, García-Cosio M, Sarhane Y, Sánchez Díaz C, Barbolla Díaz I, López Rodríguez M, Moreno MÁ, Villarubia J, Manzano L. Clinical and pathological characteristics of Castleman disease: an observational study in a Spanish tertiary hospital. Leuk Lymphoma 2019; 60:3442-3448. [PMID: 31305183 DOI: 10.1080/10428194.2019.1639168] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/09/2023]
Abstract
Castleman disease (CD) represents a heterogeneous group of lymphoproliferative disorders that share well-defined histopathological features. An observational study of patients with CD was conducted. A total of 53 patients had CD: 20 had the unicentric form (UCD) and 33 the multicentric (MCD) variant; 10 of the latter cases were infected with human herpesvirus-8 (HHV-8) and 23 were idiopathic (iMCD). Median age differed between UCD and iMCD (30 vs. 49 years, p = .004). Males were completely predominant in HHV-8-associated MCD (100%), and females were more frequent in UCD (75 vs. 48%, p = .06). Relapses were more frequent in iMCD (57 vs. 10% UCD, p = .002), and mortality was significantly higher in iMCD and the HHV-8-associated form with respect to UCD. We conclude that UCD is a benign disorder of younger ages and female predominance, while iMCD represents a different entity with more disease relapses and higher mortality.
Collapse
Affiliation(s)
- Andrés González-García
- Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. Universidad de Alcalá, IRYCIS. Madrid, Spain.,Unidad de Enfermedades Sistémicas Autoinmunes y Minoritarias. Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. IRYCIS. Madrid, Spain
| | - José Luis Patier de la Peña
- Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. Universidad de Alcalá, IRYCIS. Madrid, Spain.,Unidad de Enfermedades Sistémicas Autoinmunes y Minoritarias. Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. IRYCIS. Madrid, Spain
| | - Mónica García-Cosio
- Servicio de Anatomía Patológica, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - Yasmina Sarhane
- Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. Universidad de Alcalá, IRYCIS. Madrid, Spain
| | - Cristina Sánchez Díaz
- Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. Universidad de Alcalá, IRYCIS. Madrid, Spain
| | - Ignacio Barbolla Díaz
- Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. Universidad de Alcalá, IRYCIS. Madrid, Spain
| | - Mónica López Rodríguez
- Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. Universidad de Alcalá, IRYCIS. Madrid, Spain.,Unidad de Enfermedades Sistémicas Autoinmunes y Minoritarias. Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. IRYCIS. Madrid, Spain
| | - María Ángeles Moreno
- Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. Universidad de Alcalá, IRYCIS. Madrid, Spain
| | - Jesús Villarubia
- Servicio de Hematología. Hospital Universitario Ramón y Cajal. IRYCIS. Madrid, Spain
| | - Luis Manzano
- Servicio de Medicina Interna, Hospital Universitario Ramón y Cajal. Universidad de Alcalá, IRYCIS. Madrid, Spain
| |
Collapse
|
|
50
|
Suarez A, Reilly C, Fajgenbaum DC. Quantitative analysis of a rare disease network's international contact database and E-repository provides insights into biobanking in the electronic consent era. Orphanet J Rare Dis 2019; 14:173. [PMID: 31296233 PMCID: PMC6625003 DOI: 10.1186/s13023-019-1145-y] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2019] [Accepted: 06/25/2019] [Indexed: 01/09/2023] Open
Abstract
BACKGROUND Castleman disease (CD) describes a group of rare and poorly understood lymphoproliferative disorders that include unicentric CD (UCD), Human Herpes Virus-8 (HHV8)-associated multicentric CD (HHV8 + MCD), and HHV8-negative/idiopathic MCD (iMCD). Efforts to advance research and drug discovery for CD have been slowed by challenges shared by other rare diseases, such as collecting and centralizing data and biospecimens for research. To collect disease characteristic data and identify individuals interested in contributing biospecimens for research, a global research organization - the Castleman Disease Collaborative Network (CDCN) - established an international Contact Database and electronic repository (E-repository). Herein, we performed analyses of these datasets to further characterize CD and gain insights into research biospecimen acquisition. RESULTS Descriptive statistical analyses were performed on 891 participants from the Contact Database and 166 patients in the E-repository. The median age of patients at the time of enrollment in the Contact Database and E-repository was 42 ± 15.7 and 35 ± 14.8, respectively. The E-repository had increased representation from patients with MCD and the iMCD subtype compared to other sub-groups. Though the majority of participants were from the USA, a total of 49 countries on 6 continents were represented. Several patient characteristics in the Contact Database were associated with subsequent enrollment in the E-repository. There were significantly more MCD patients (p < 0.0001) and females (p = 0.002) enrolled in the E-repository compared to the Contact Database. Patient's year of birth, date of registration, preferred method of communication, and relationship to the patient were also significantly associated with enrollment in the e-Repository. CONCLUSIONS This study of the largest- dataset of CD patients worldwide provides insights into disease phenotypes, characteristics of patients interested in contributing data and biospecimens for research, and methods for successfully acquiring data and biospecimens. Generally, the factors associated with enrollment in the E-repository represented severity of disease subtype, proximity to the research, and patient motivation. We hope that these findings and the sample documentation (e.g., electronic consent, recruitment materials) provided with this article will assist future rare disease efforts with overcoming hurdles.
Collapse
Affiliation(s)
- Alexander Suarez
- Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA USA
| | - Curran Reilly
- Castleman Disease Collaborative Network, Philadelphia, PA USA
| | - David C. Fajgenbaum
- Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA USA
| |
Collapse
|