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Ogunnaya F, Kambal ME, Ali TE, Elsayed D, Bustillo J. Unmasking POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome in a Heart Failure Patient: A Diagnostic Challenge. Cureus 2025; 17:e81075. [PMID: 40271327 PMCID: PMC12017233 DOI: 10.7759/cureus.81075] [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] [Accepted: 03/24/2025] [Indexed: 04/25/2025] Open
Abstract
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome remains poorly understood. It is a paraneoplastic syndrome caused by underlying plasma cell dyscrasia, and there is uncertainty about the features required to establish the diagnosis, treatment efficacy, and prognosis. Almost all patients have polyneuropathy or plasma cell disorder, and approximately one-third have Castleman's disease. Peripheral neuropathy often serves as the primary presenting complaint. While extensive discussions exist regarding POEMS syndrome diagnosis and treatment advancements, literature reports of heart failure symptoms associated with POEMS syndrome are scarce. In this article, we present a case of newly diagnosed POEMS syndrome in a 47-year-old man who initially presented with bilateral lower extremities edema and weakness. Although the initial workup was suggestive of heart failure, subsequent evaluation revealed monoclonal gammopathy, sclerotic bone lesions, and Castleman's disease, ultimately leading to the diagnosis of POEMS syndrome.
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Affiliation(s)
- Frances Ogunnaya
- Internal Medicine, Newark Beth Israel Medical Center, Newark, USA
| | | | - Thowaiba E Ali
- Internal Medicine, University of Khartoum, Khartoum, SDN
- Healthcare Administration, University of Tennessee at Chattanooga, Chattanooga, USA
| | - Dina Elsayed
- Internal Medicine, Newark Beth Israel Medical Center, Newark, USA
| | - Jose Bustillo
- Medicine-Pediatrics, Newark Beth Israel Medical Center, Newark, USA
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2
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Becker B, Stino A. Top 10 Clinical Pearls in Paraproteinemic Neuropathies. Semin Neurol 2025; 45:99-111. [PMID: 39419069 DOI: 10.1055/s-0044-1791769] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2024]
Abstract
Paraproteinemic neuropathies represent an important subset of peripheral neuropathies. Once identified, further evaluation into the paraproteinemic subtype, clinical exam pattern, and electrodiagnostic phenotype helps clarify if the paraproteinemia is coincidental or causal of the neuropathy, as not all paraproteinemias cause neuropathy. Of all paraproteinemias, immunoglobulin M (IgM)-associated peripheral neuropathy, or IgM neuropathy, is of particular importance as half of IgM neuropathies also harbor anti-myelin-associated glycoprotein antibodies, which produce a characteristic demyelinating pattern on nerve conduction testing. Immunoglobulin G and immunoglobulin A paraproteinemias are less strongly associated with peripheral neuropathy, except in the setting of multiple myeloma or osteosclerotic myeloma (POEMS syndrome), which have characteristic systemic features. In multiple myeloma, chemotherapy is more likely to result in neuropathy than the myeloma itself. Finally, the presence of systemic features (e.g., cardiomyopathy, nephropathy, recurrent carpal tunnel syndrome, and autonomic insufficiency) should raise concern for hereditary or acquired light (AL) chain amyloidosis. AL amyloidosis can occur in the setting of any light or heavy chain paraproteinemia. Central to the proper evaluation of paraproteinemic neuropathy is electrodiagnostic testing, which helps delineate axonal versus demyelinating paraproteinemic neuropathy, the latter often misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy.
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Affiliation(s)
- Benjamin Becker
- Division of Neuromuscular Medicine, Department of Neurology, University of Michigan, Ann Arbor, Michigan
| | - Amro Stino
- Division of Neuromuscular Medicine, Department of Neurology, University of Michigan, Ann Arbor, Michigan
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Liu W, Zhou Y, Li L, Zhang F, Xiong Z, Hou S. A case report of an M protein-negative patient with POEMS syndrome associated with renal involvement. BMC Nephrol 2024; 25:472. [PMID: 39719549 DOI: 10.1186/s12882-024-03898-z] [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: 07/21/2024] [Accepted: 11/28/2024] [Indexed: 12/26/2024] Open
Abstract
BACKGROUND POEMS syndrome with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes is an uncommon plasma cell paraneoplastic syndrome involving multiple system. It is relatively rare in clinical practice, and renal involvement is a usual yet easily overlooked symptom. CASE PRESENTATION We successfully treated a patient with M protein-negative POEMS syndrome with membranoproliferative glomerulonephritis (MPGN) findings and thrombotic microangiopathic changes by comparing the level of Vascular endothelial growth factor (VEGF) in the serum and the changes in polyserositis before and after the patient's treatment. CONCLUSION POEMS syndrome clinically involves multiple systems and has complex symptoms. Because of the diversity of the disease manifestations, identification of atypical POEMS syndrome and timely intervention are important for patient survival and prognosis.
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Affiliation(s)
- Wenlin Liu
- Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, 518000, China
| | - Yue Zhou
- Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, 518000, China
| | - Lingyan Li
- Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, 518000, China
| | - Fan Zhang
- Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, 518000, China
| | - Zuying Xiong
- Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, 518000, China
| | - Shuang Hou
- Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, 518000, China.
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Tan S, Shang M, Masaki Y, Ni J, Da Y, Sun J, Cao Y, Su L, Sun W. Case report: Atypical POEMS syndrome without polyneuropathy, complicated by borderline TAFRO syndrome. Front Med (Lausanne) 2024; 11:1445971. [PMID: 39720653 PMCID: PMC11666388 DOI: 10.3389/fmed.2024.1445971] [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: 06/08/2024] [Accepted: 11/15/2024] [Indexed: 12/26/2024] Open
Abstract
POEMS syndrome is a complex and rare hematological disease involving multiple physiological systems. According to the currently accepted diagnostic criteria for POEMS syndrome, polyneuropathy is one of the primary mandatory criteria. A patient presented with M protein, Castleman disease (CD), elevated vascular endothelial growth factor (VEGF), extravascular volume overload, and endocrinopathy. However, edema of the extremities hindered the diagnosis of polyneuropathy by electromyography (EMG). Eventually, we diagnosed the patient with atypical POEMS syndrome without polyneuropathy. The case also exhibited features consistent with TAFRO syndrome, such as anasarca, thrombocytopenia, and renal insufficiency. This underscores the need to emphasize that TAFRO syndrome is principally a systemic inflammatory disorder. Timely diagnosis and treatment with dexamethasone, followed by several sessions of lenalidomide and dexamethasone (Rd) regimen chemotherapy, resulted in complete remission (CR), and was followed by autologous stem cell transplantation (ASCT). This case offers valuable insights into the diagnosis and treatment of POEMS syndrome, which may prompt a reconsideration of the diagnostic criteria for this syndrome.
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Affiliation(s)
- Shuai Tan
- Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China
| | - Mingyue Shang
- Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China
| | - Yasufumi Masaki
- Hematology and Immunology, Kanazawa Medicai University, Uchinada, Japan
| | - Jing Ni
- Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China
| | - Yuwei Da
- Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China
| | - Jing Sun
- Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China
| | - Yaofang Cao
- Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China
| | - Li Su
- Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China
| | - Wanling Sun
- Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China
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Zambrano Zambrano K, Martinez Salazar J, Velarde Michaud JC, Montalvo Lopez Gavito A, Zambrano Zambrano A. Unraveling Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes Syndrome: Diagnostic Challenges and Therapeutic Strategies at a National Tertiary Care Center. Cureus 2024; 16:e75620. [PMID: 39803068 PMCID: PMC11725331 DOI: 10.7759/cureus.75620] [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] [Accepted: 12/12/2024] [Indexed: 01/16/2025] Open
Abstract
Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) syndrome is a rare paraneoplastic disorder caused by plasma cell proliferation and overproduction of cytokines, particularly vascular endothelial growth factor (VEGF). This complex syndrome affects multiple organ systems and presents with a broad range of clinical and laboratory manifestations, which can complicate both diagnosis and management. Not all components of the acronym are observed in every patient, highlighting the clinical heterogeneity of the condition. The case discussed herein illustrates the diagnostic approach to a patient suspected of having POEMS syndrome.
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Zhao B, Zhou X, Zheng P, Zhang B, Feng X, Chen J, Cai L, Chen Y, He L, Su J, Cheng S, Zeng Y, Li G, Ji B, Wu J, Feng W, Liu M, Jin Y, Liu T, Mo X, Wu J, Wu H, Zhang H, Zheng Z, Zheng Z, Sun J, Li Y. Expert consensus on the off-label use in China of drugs for rare hematologic diseases (2024 edition). Front Pharmacol 2024; 15:1477550. [PMID: 39650164 PMCID: PMC11621627 DOI: 10.3389/fphar.2024.1477550] [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: 08/08/2024] [Accepted: 09/17/2024] [Indexed: 12/11/2024] Open
Abstract
Drug package inserts are a crucial foundation for clinical medication practices and serve as the legal basis for guiding rational drug use and ensuring patient safety and efficacy. As rare disease treatments evolve, current package inserts often need to meet the clinical requirements for treating such conditions, frequently resulting in off-label drug use. This consensus is derived from discussions between Guangdong Pharmaceutical Association Hematologic Rare Diseases Group experts. The consensus aims to provide a framework and reference for the clinical application of off-label drug use in treating rare hematologic diseases.
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Affiliation(s)
- Boxin Zhao
- Department of Pharmacy, Nanfang Hospital, Southern Medical University, Guangzhou, China
- Clinical Pharmacy Center, Nanfang Hospital, Southern Medical University, Guangzhou, China
| | - Xuan Zhou
- Department of Hematology, Nanfang Hospital, Southern Medical University, Guangzhou, China
| | - Ping Zheng
- Department of Pharmacy, Nanfang Hospital, Southern Medical University, Guangzhou, China
- Clinical Pharmacy Center, Nanfang Hospital, Southern Medical University, Guangzhou, China
| | - Bo Zhang
- Department of Pharmacy, Peking Union Medical College Hospital, Beijing, China
- State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Beijing, China
| | - Xiaoqin Feng
- Department of Pediatrics, Nanfang Hospital, Southern Medical University, Guangzhou, China
| | - Jie Chen
- Department of Pharmacy, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China
| | - Lisheng Cai
- Department of Hematology, Shenzhen Second People’s Hospital, Shenzhen, China
| | - Yilu Chen
- Department of Pharmacy, Guangzhou Women and Children’s Medical Center, Guangzhou, China
| | - Liya He
- Department of Pediatrics, Guangzhou Women and Children’s Medical Center, Guangzhou, China
| | - Jianfen Su
- Department of Pharmacy, Panyu Central Hospital, Guangzhou, China
| | - Shuqin Cheng
- Department of Hematology, Panyu Central Hospital, Guangzhou, China
| | - Yingtong Zeng
- Department of Pharmacy, Guangdong Provincial People’s Hospital, Southern Medical University, Guangzhou, China
| | - Guowei Li
- Department of Hematology, Huizhou Central People’s Hospital, Huizhou, China
| | - Bo Ji
- Department of Clinical Pharmacy, General Hospital of Southern Theatre Command of PLA, Guangzhou, China
| | - Jianlong Wu
- Department of Pharmacy, Shenzhen Second People’s Hospital, Shenzhen, China
| | - Weiyi Feng
- Department of Pharmacy, The First Affiliated Hospital of Xi’an Jiaotong University, Xi’an, Shaanxi, China
| | - Maobai Liu
- Department of Pharmacy, Fujian Medical University Union Hospital, Fuzhou, China
| | - Yiran Jin
- Department of Pharmacy, The Second Hospital of Hebei Medical University, Shijiazhuang, China
| | - Taotao Liu
- Pharmacy Department, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Xiaolan Mo
- Department of Pharmacy, Guangzhou Women and Children’s Medical Center, Guangzhou, China
| | - Junyan Wu
- Department of Pharmacy, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China
| | - Hui Wu
- Department of Pharmacy, First Affiliated Hospital of Kunming Medical University, Kunming, China
| | - Hongliang Zhang
- Pharmacy Department, The First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Zhichang Zheng
- Department of Pharmacy, The Affiliated Hospital of Guizhou Medical University, Guiyang, China
| | - Zhihua Zheng
- Guangdong Pharmaceutical Association, Guangzhou, China
| | - Jing Sun
- Department of Hematology, Nanfang Hospital, Southern Medical University, Guangzhou, China
| | - Yilei Li
- Department of Pharmacy, Nanfang Hospital, Southern Medical University, Guangzhou, China
- Clinical Pharmacy Center, Nanfang Hospital, Southern Medical University, Guangzhou, China
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Rodriguez-Domínguez V, Hernández-García BJ, Abenza-Abildúa MJ. [Hydrocephalus associated with POEMS syndrome]. Med Clin (Barc) 2024; 163:212-213. [PMID: 38697895 DOI: 10.1016/j.medcli.2024.02.022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/23/2024] [Revised: 02/13/2024] [Accepted: 02/14/2024] [Indexed: 05/05/2024]
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He H, Hou N, Chen X, Song Y, Qiang W, Liu J, Lu J, Fu W, Du J. An open-label, prospective trial to evaluate the efficacy and safety of ixazomib in combination with cyclophosphamide and dexamethasone in patients with newly diagnosed POEMS syndrome. Br J Haematol 2024; 205:478-482. [PMID: 38955502 DOI: 10.1111/bjh.19626] [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: 01/01/2024] [Accepted: 06/24/2024] [Indexed: 07/04/2024]
Abstract
This open-label, prospective trial evaluated the combination of ixazomib, cyclophosphamide and dexamethasone (ICD) in 12 newly diagnosed POEMS syndrome patients. The study is registered with the Chinese Clinical Trials Registry (ChiCTR2000030072). The treatment protocol consisted of 12 cycles of the ICD regimen compromising ixazomib (4 mg on Days 1, 8 and 15), oral cyclophosphamide (300 mg on Days 1, 8 and 15) and dexamethasone (20 mg weekly). A total of 12 patients received a median of 10 (range: 3-23) cycles of the ICD regimen. The haematological response could be evaluated in 10 patients. The overall haematological response rate was 80% (8/10), with 30% (3/10) achieving complete haematological response, and the overall serum VEGF response rate and neurological response were 100% and 83.3% respectively. Two patients experienced grade 3/4 AEs, including diarrhoea (n = 1) and leukopenia (n = 1). The combination of ixazomib, cyclophosphamide and dexamethasone demonstrated both efficacy and safety in newly diagnosed POEMS syndrome, making it a viable treatment option.
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Affiliation(s)
- Haiyan He
- Department of Hematology, Myeloma and Lymphoma Center, Changzheng Hospital, Shanghai, China
| | - Nan Hou
- Department of Hematology, Myeloma and Lymphoma Center, Changzheng Hospital, Shanghai, China
| | - Xi Chen
- Department of Hematology, Myeloma and Lymphoma Center, Changzheng Hospital, Shanghai, China
| | - Yaqi Song
- Department of Hematology, Myeloma and Lymphoma Center, Changzheng Hospital, Shanghai, China
| | - Wanting Qiang
- Department of Hematology, Myeloma and Lymphoma Center, Changzheng Hospital, Shanghai, China
| | - Jin Liu
- Department of Hematology, Myeloma and Lymphoma Center, Changzheng Hospital, Shanghai, China
| | - Jing Lu
- Department of Hematology, Myeloma and Lymphoma Center, Changzheng Hospital, Shanghai, China
| | - Weijun Fu
- Department of Hematology, Myeloma and Lymphoma Center, Changzheng Hospital, Shanghai, China
| | - Juan Du
- Department of Hematology, Myeloma and Lymphoma Center, Changzheng Hospital, Shanghai, China
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Khouri J. Ixazomib in POEMS syndrome: 'Ixa'ctly what we need? Br J Haematol 2024; 205:418-419. [PMID: 39004099 DOI: 10.1111/bjh.19645] [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: 07/02/2024] [Accepted: 07/04/2024] [Indexed: 07/16/2024]
Abstract
The role of the proteasome inhibitor ixazomib in the treatment of POEMS syndrome continues to evolve. He and colleagues present the results of a study investigating ixazomib in combination with cyclophosphamide and dexamethasone in newly diagnosed POEMS patients. The triplet showed excellent efficacy and tolerability, and constitutes an effective treatment option for patients with POEMS. Commentary on: He et al. An open-label, prospective trial to evaluate the efficacy and safety of ixazomib in combination with cyclophosphamide and dexamethasone in patients with newly-diagnosed POEMS syndrome. Br J Haematol 2024;205:478-482.
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Affiliation(s)
- Jack Khouri
- Department of Hematology and Medical Oncology, Taussig Cancer Center, Cleveland Clinic, Cleveland, Ohio, USA
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Adeuyan O, Magro CM, Trager MH, Gordon ER, Lapolla BA, Schreidah CM, Fahmy LM, Geskin LJ. Plasma cell and neutrophil enriched neovascularization with granulomatous lymphangitis in POEMS syndrome. JAAD Case Rep 2024; 49:125-128. [PMID: 39036500 PMCID: PMC11260350 DOI: 10.1016/j.jdcr.2024.03.025] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/23/2024] Open
Affiliation(s)
- Oluwaseyi Adeuyan
- Columbia University Vagelos College of Physicians and Surgeons, New York, New York
| | - Cynthia M. Magro
- Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York
| | - Megan H. Trager
- Department of Dermatology, Columbia University Irving Medical Center, New York, New York
| | - Emily R. Gordon
- Columbia University Vagelos College of Physicians and Surgeons, New York, New York
| | - Brigit A. Lapolla
- Department of Dermatology, Columbia University Irving Medical Center, New York, New York
| | - Celine M. Schreidah
- Columbia University Vagelos College of Physicians and Surgeons, New York, New York
| | - Lauren M. Fahmy
- Columbia University Vagelos College of Physicians and Surgeons, New York, New York
| | - Larisa J. Geskin
- Columbia University Vagelos College of Physicians and Surgeons, New York, New York
- Department of Dermatology, Columbia University Irving Medical Center, New York, New York
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Li ZY, Chen JJ, Lu FY, Gan MF, Tung TH, Hong D. Non-POEMS osteosclerotic multiple myeloma: Clinical characteristics and differential diagnosis. J Bone Oncol 2024; 45:100595. [PMID: 38572352 PMCID: PMC10987900 DOI: 10.1016/j.jbo.2024.100595] [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: 01/11/2024] [Revised: 03/15/2024] [Accepted: 03/18/2024] [Indexed: 04/05/2024] Open
Abstract
Osteosclerosis in multiple myeloma (MM) is typically associated with rare POEMS syndrome, characterized by polyneuropathy (P), organomegaly (O), endocrinopathy (E), M-protein (M), and skin changes (S). However, osteosclerosis in multiple myeloma (MM) without POEMS syndrome, defined as non-POEMS Osteosclerotic MM, is exceedingly rare. We report a 70-year-old man with rib pain, remarkably high bone mineral density and diffuse osteosclerosis. The diagnosis of non-POEMS osteosclerotic MM was confirmed by biopsy and aspiration of bone marrow through surgery. A literature review spanning from 1990 identified 12 cases of similar non-POEMS osteosclerotic MM, including 5 males and 7 females with a mean age of 59.7 ± 10.6 years. The non-POEMS osteosclerotic MM can be divided into two subtypes, the osteosclerotic lesion subtype and the diffuse osteosclerosis subtype. Absence of polyneuropathy and organomegaly are the main factors that differentiate non-POEMS osteosclerotic MM from POEMS. A hyperactive osteoblastic process might be the etiology of diffuse osteosclerosis. Further research is needed to understand its etiology and pathophysiology.
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Affiliation(s)
- Zi-Yan Li
- Department of Bone Disease, Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University, Linhai, China
| | - Jiang-Jie Chen
- Department of Bone Disease, Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University, Linhai, China
- Department of Orthopedics, Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University, Linhai, China
| | - Fang-Ying Lu
- Department of Bone Disease, Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University, Linhai, China
- Department of Orthopedics, Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University, Linhai, China
| | - Mei-Fu Gan
- Department of Pathology, Taizhou Hospital affiliated to Wenzhou Medical University, Linhai, China
| | - Tao-Hsin Tung
- Evidence-Based Medicine Center, Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University, Linhai, China
| | - Dun Hong
- Department of Bone Disease, Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University, Linhai, China
- Bone Metabolism and Development Research Center, Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University, Linhai, China
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Boulanger MC, Tieger MG, Eliott D, Yee AJ. Intravitreal aflibercept confounds interpretation of plasma VEGF (vascular endothelial growth factor) levels in POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome. EJHAEM 2024; 5:421-422. [PMID: 38633109 PMCID: PMC11020120 DOI: 10.1002/jha2.883] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/17/2024] [Revised: 03/11/2024] [Accepted: 03/13/2024] [Indexed: 04/19/2024]
Affiliation(s)
- Mary C. Boulanger
- Massachusetts General Hospital Cancer CenterBostonMassachusettsUSA
- Harvard Medical SchoolBostonMassachusettsUSA
| | - Marisa G. Tieger
- Massachusetts Eye and Ear InfirmaryBostonMassachusettsUSA
- Present address:
New England Eye Center, Tufts Medical Center
| | - Dean Eliott
- Harvard Medical SchoolBostonMassachusettsUSA
- Massachusetts Eye and Ear InfirmaryBostonMassachusettsUSA
| | - Andrew J. Yee
- Massachusetts General Hospital Cancer CenterBostonMassachusettsUSA
- Harvard Medical SchoolBostonMassachusettsUSA
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Li JR, Feng LY, Li JW, Liao Y, Liu FQ. Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome with dilated cardiomyopathy: A case report. World J Clin Cases 2024; 12:601-606. [PMID: 38322456 PMCID: PMC10841945 DOI: 10.12998/wjcc.v12.i3.601] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/11/2023] [Revised: 12/16/2023] [Accepted: 01/04/2024] [Indexed: 01/18/2024] Open
Abstract
BACKGROUND Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare paraneoplastic syndrome that encompass multiple systems. The most common clinical symptoms of POEMS syndrome are progressive sensorimotor polyneuropathy, organ enlargement, endocrine disorders, darkening skin, a monoclonal plasma cell proliferative disorder, and lymph node hyperplasia. The organomegaly consists of hepatosplenomegaly and/or lymphadenopathy; cases of cardiomyopathy are rare. Diagnoses are often delayed because of the atypical nature of the syndrome, exposing patients to possibly severe disability. Therefore, identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients. CASE SUMMARY Herein, we report the case of a 59-year-old woman with POEMS syndrome that involved dilated cardiomyopathy. The patient presented to the hospital with complaints of shortness of breath and discomfort in the chest. The patient reported previous experiences of limb numbness. During hospitalization, the brain natriuretic peptide levels were 3504.0 pg/mL. Color doppler echocardiography showed an enlarged left side of the heart, along with ventricular wall hypokinesis and compromised functioning of the same side of the heart. Abdominal color ultrasonography revealed that the patient's spleen was enlarged. Observations from cardiac magnetic resonance imaging showed that the left side of the heart was enlarged. Slight myocardical fibrosis was also observed. Electromyography was described as a symmetric sensorimotor demyelinating polyneuropathy. Further immunoelectrophoresis of the serum showed the presence of a monoclonal IGA λ M protein. The vascular endothelial growth factor levels were 622.56 pg/mL. Flow cytometric and immunohistochemical staining of the bone marrow detected no monoclonal plasma cells. Finally, the patient was diagnosed with POEMS syndrome associated with dilated cardiomyopathy. The chest-related discomfort and the shortness of breath resolved after the administration of lenalidomide and dexamethasone. CONCLUSION When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin, the POEMS syndrome is the most possible diagnosis.
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Affiliation(s)
- Jia-Rong Li
- Department of General Medicine, Xiangtan Central Hospital, Xiangtan 411100, Hunan Province, China
| | - Lei-Yu Feng
- Department of General Medicine, Xiangtan Central Hospital, Xiangtan 411100, Hunan Province, China
| | - Jian-Wei Li
- Department of General Medicine, Xiangtan Central Hospital, Xiangtan 411100, Hunan Province, China
| | - Yu Liao
- Department of General Medicine, Xiangtan Central Hospital, Xiangtan 411100, Hunan Province, China
| | - Fei-Qi Liu
- Department of General Medicine, Xiangtan Central Hospital, Xiangtan 411100, Hunan Province, China
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Briani C, Visentin A. Hematologic malignancies and hematopoietic stem cell transplantation. HANDBOOK OF CLINICAL NEUROLOGY 2024; 200:419-429. [PMID: 38494294 DOI: 10.1016/b978-0-12-823912-4.00016-5] [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: 03/19/2024]
Abstract
Paraneoplastic neurologic syndromes are rarely associated with hematologic malignancies. In their rarity, lymphomas are the diseases with more frequent paraneoplastic neurologic syndrome. High-risk antibodies are absent in most lymphoma-associated paraneoplastic neurologic syndromes, with the exception of antibodies to Tr/DNER in paraneoplastic cerebellar degeneration, mGluR5 in limbic encephalitis, and mGluR1 in some cerebellar ataxias. Peripheral nervous system paraneoplastic neurologic syndromes are rare and heterogeneous, with a prevalence of demyelinating polyradiculoneuropathy in non-Hodgkin lymphoma. Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) is a rare, paraneoplastic syndrome due to an underlying plasma cell disorder. The diagnosis is based on defined criteria, and vascular endothelial growth factor (VEGF), not an antibody, is considered a reliable diagnostic marker that also mirrors therapy response. As with the paraneoplastic neurologic syndromes in solid tumors, therapies rely on cancer treatment associated with immunomodulatory treatment with better response in PNS with antibodies to surface antigens. The best outcome is generally present in Ophelia syndrome/limbic encephalitis with anti-mGluR5 antibodies, with frequent complete recovery. Besides patients with isolated osteosclerotic lesions (where radiotherapy is indicated), hematopoietic stem-cell transplantation is the therapy of choice in patients with POEMS syndrome. In the paraneoplastic neurologic syndromes secondary to immune checkpoint inhibitors, discontinuation of the drug together with immunomodulatory treatment is recommended.
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Affiliation(s)
- Chiara Briani
- Department of Neurosciences, Neurology Unit, University of Padova, Padova, Italy.
| | - Andrea Visentin
- Department of Medicine, Hematology Unit, University of Padova, Padova, Italy
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15
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Huang Y, Luo Y, Hou H, Gao J. Pulmonary manifestations of POEM syndrome: a retrospective analysis of 282 cases. BMC Pulm Med 2023; 23:465. [PMID: 37993859 PMCID: PMC10666414 DOI: 10.1186/s12890-023-02741-9] [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: 09/07/2023] [Accepted: 10/30/2023] [Indexed: 11/24/2023] Open
Abstract
BACKGROUND Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic clonal plasma cell disorder. Pulmonary involvement is frequently found in patients with POEMS syndrome, manifesting various clinical features. Therefore, to improve diagnostic accuracy and provide treatment strategies, a comprehensive analysis of pulmonary manifestations of POEMS syndrome is needed. METHODS This retrospective study included patients with POEMS syndrome at Peking Union Medical College Hospital, a major referral medical center in China, between June 1, 2013, and June 1, 2023. Demographic data, laboratory findings, pulmonary function test results, echocardiograms, and chest imaging data were extracted. Continuous variables were compared using the t-test or Mann-Whitney method. Pearson's chi-square test or Fisher's exact test was conducted to compare categorical data. RESULTS Overall, 282 individuals diagnosed with POEMS syndrome were included in this study, of which 56% were male with an average age of 48.7 years. Respiratory symptoms were found in 40.1% of the patients, with dyspnea as the most common symptom (34.4%). Chest computed tomography and echocardiography findings showed that 56.4% of patients exhibited pleural effusion, 62.8% displayed mediastinal or hilar lymphadenopathy, 46.5% presented pleural thickening, 27.3% demonstrated bone lesions of the ribs or thoracic vertebra, 7.8% showed lung interstitial abnormalities, and 35.5% had pulmonary hypertension. Decreased diffuse capacity and restrictive ventilatory patterns were identified in 85.2% (115 cases) and 47.4% (64 cases) of patients, respectively. Patients with respiratory symptoms exhibited higher declined lung function measures than those having no respiratory symptoms. High-risk patients with poor prognosis showed more pulmonary function abnormalities. CONCLUSION Abnormalities in pulmonary manifestations constitute the significant features of POEMS syndrome. Several patients with POEMS syndrome presented with respiratory symptoms at the initial evaluation. These findings underscore the importance of early identification and accurate diagnosis of POEMS syndrome by clinicians, particularly in cases involving lung and multisystem.
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Affiliation(s)
- Yilin Huang
- Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China
| | - Yang Luo
- Peking University HuiLongGuan Clinical Medical School, Beijing HuiLongGuan Hospital, Beijing, 100096, People's Republic of China
| | - Huan Hou
- Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China
| | - Jinming Gao
- Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
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16
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Dima D, Valent J, Khouri J. Daratumumab monotherapy for relapsed POEMS syndrome. Am J Hematol 2023; 98:E291-E294. [PMID: 37548551 DOI: 10.1002/ajh.27050] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/01/2023] [Revised: 07/13/2023] [Accepted: 07/21/2023] [Indexed: 08/08/2023]
Affiliation(s)
- Danai Dima
- Department of Hematology and Medical Oncology, Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Jason Valent
- Department of Hematology and Medical Oncology, Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Jack Khouri
- Department of Hematology and Medical Oncology, Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio, USA
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17
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Zhang F, Wu Z, Sun S, Fu Y, Chen Y, Liu J. POEMS syndrome in the 21st century: A bibliometric analysis. Heliyon 2023; 9:e20612. [PMID: 37842561 PMCID: PMC10570589 DOI: 10.1016/j.heliyon.2023.e20612] [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: 07/03/2023] [Revised: 09/26/2023] [Accepted: 10/02/2023] [Indexed: 10/17/2023] Open
Abstract
BACKGROUND POEMS syndrome is a rare and complex plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, high M protein levels, and skin changes. Understanding of POEMS syndrome has advanced rapidly since the 21st century. This study aims to summarize and evaluate the research status of POEMS syndrome in the past 23 years through a bibliometric and visualization analysis, and identify research focuses and emerging hotspots for the future. METHODS POEMS syndrome-related articles published between January 1, 2000, and March 8, 2023, were systematically retrieved from the Web of Science Core Collection. Data processing and visualization analysis were carried out using a combination of R software, HistCite, VOSviewer, and CiteSpace. RESULTS Since entering the 21st century, 3677 authors from 1125 institutions in 68 countries/regions have published 830 original and review articles on POEMS syndrome in 408 journals so far, among which the USA, Japan, and China published the most articles, and Mayo Clinic, Udice French Research Universities, and Peking Union Medical College listed the top three most prolific institutions. However, collaborative research across countries and groups in the study of POEMS syndrome remain significantly limited. Angela Dispenzieri ranked first in POEMS syndrome research from every aspect of authors, producing the most papers and contributing the most-cited article, followed by Satoshi Kuwabara and Sonoko Misawa. Internal medicine was the most productive journal on POEMS syndrome. "endothelial growth factor" was the keyword with the highest occurrence except for "POEMS syndrome", and "bevacizumab", "lenalidomide", "dexamethasone", and "management" were recognized as emerging topics. CONCLUSION This study utilized bibliometric and visualization analysis to systematically summarize the research of POEMS syndrome in the first two decades of the 21st century, offering a data-based and objective perspective on the field of POEMS syndrome and guiding researchers in the identification of novel research directions.
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Affiliation(s)
- Fangrong Zhang
- Department of Hematology, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China
| | - Zhimin Wu
- Guiyang maternal and child health care hospital, Guiyang Children's Hospital, Guiyang, Guizhou, 550003, China
| | - Shanyi Sun
- Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, 410008, China
| | - Yunfeng Fu
- Department of Blood Transfusion, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China
| | - Yi Chen
- Department of Breast and Thyroid Surgery, The Afflicted Cancer Hospital of Xinjiang Medical University, Urumqi, Xinjiang Uygur Autonomous Region, 830011, China
- The China-US (Henan) Hormel Cancer Institute, Zhengzhou, Henan, 450008, China
| | - Jing Liu
- Department of Hematology, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China
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18
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Beydoun SR, Darki L. Paraproteinemic Neuropathies. Continuum (Minneap Minn) 2023; 29:1492-1513. [PMID: 37851040 DOI: 10.1212/con.0000000000001294] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2023]
Abstract
OBJECTIVE Coexistence of polyneuropathy and gammopathy is a common but potentially challenging situation in clinical practice. This article reviews the clinical, electrophysiologic, and hematologic phenotypes of the paraproteinemic neuropathies and the diagnostic and treatment strategies for each. LATEST DEVELOPMENTS Advances in our understanding of the underlying pathophysiology of various paraproteinemic neuropathies and their corresponding phenotypes have identified potential new therapeutic targets. Therapeutic strategies to diminish anti-myelin-associated glycoprotein (MAG) IgM antibodies have shown partial and inconsistent efficacy; however, antigen-specific immune therapy is being investigated as a novel treatment to remove the presumably pathogenic anti-MAG antibody. Advances in genetic and cell signaling studies have resulted in the approval of Bruton tyrosine kinase inhibitors for Waldenström macroglobulinemia. Monoclonal antibodies are being investigated for the treatment of light chain amyloidosis. ESSENTIAL POINTS Early recognition and treatment of underlying plasma cell disorders improves clinical outcomes in patients with paraproteinemic neuropathy. Despite significant progress, our knowledge regarding underlying mechanisms for paraproteinemic neuropathy is still limited. Clinicians' awareness of clinical phenotypes, electrophysiologic hallmarks, and hematologic findings of the different paraproteinemic neuropathies is crucial to promptly identify and treat patients and to avert misdiagnosis. Multidisciplinary collaboration among specialists, including neurologists and hematologists, is paramount for the optimal treatment of these patients with overlapping conditions.
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19
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Malhotra B, Sood A, Malhotra P, Bhadada S. POEMS syndrome: an unusual cause of exuberant callus formation. BMJ Case Rep 2023; 16:e252891. [PMID: 37699737 PMCID: PMC10503389 DOI: 10.1136/bcr-2022-252891] [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] [Indexed: 09/14/2023] Open
Abstract
Several growth factors are expressed in distinct temporal and spatial patterns during fracture repair. Together, they lead to angiogenesis-osteogenesis coupling and hence bone healing by callus formation. Of these growth factors, vascular endothelial growth factor is of particular interest because of its ability to induce neovascularisation (angiogenesis) and osteoclast invasion in soft callus. Hyperplastic callus can be seen in metabolic bone disease, endocrine disease and in patients with traumatic brain injury, but has not been described so far in polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome. This report is a case of POEMS syndrome with unusual presentation in the form of exuberant callus formation at fracture sites.
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Affiliation(s)
- Bhanu Malhotra
- Endocrinology, PGIMER, Chandigarh, Haryana and Punjab, India
| | - Ashwini Sood
- Nuclear medicine, PGIMER, Chandigarh, Haryana and Punjab, India
| | - Pankaj Malhotra
- Hematology, Internal Medicine, PGIMER, Chandigarh, Haryana and Punjab, India
| | - Sanjay Bhadada
- Endocrinology, PGIMER, Chandigarh, Haryana and Punjab, India
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20
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Dziadkowiak E, Nowakowska-Kotas M, Rałowska-Gmoch W, Budrewicz S, Koszewicz M. Molecular, Electrophysiological, and Ultrasonographic Differences in Selected Immune-Mediated Neuropathies with Therapeutic Implications. Int J Mol Sci 2023; 24:ijms24119180. [PMID: 37298132 DOI: 10.3390/ijms24119180] [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: 03/31/2023] [Revised: 05/16/2023] [Accepted: 05/19/2023] [Indexed: 06/12/2023] Open
Abstract
The spectrum of immune-mediated neuropathies is broad and the different subtypes are still being researched. With the numerous subtypes of immune-mediated neuropathies, establishing the appropriate diagnosis in normal clinical practice is challenging. The treatment of these disorders is also troublesome. The authors have undertaken a literature review of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), Guillain-Barre syndrome (GBS) and multifocal motor neuropathy (MMN). The molecular, electrophysiological and ultrasound features of these autoimmune polyneuropathies are analyzed, highlighting the differences in diagnosis and ultimately treatment. The immune dysfunction can lead to damage to the peripheral nervous system. In practice, it is suspected that these disorders are caused by autoimmunity to proteins located in the node of Ranvier or myelin components of peripheral nerves, although disease-associated autoantibodies have not been identified for all disorders. The electrophysiological presence of conduction blocks is another important factor characterizing separate subgroups of treatment-naive motor neuropathies, including multifocal CIDP (synonyms: multifocal demyelinating neuropathy with persistent conduction block), which differs from multifocal motor neuropathy with conduction block (MMN) in both responses to treatment modalities and electrophysiological features. Ultrasound is a reliable method for diagnosing immune-mediated neuropathies, particularly when alternative diagnostic examinations yield inconclusive results. In overall terms, the management of these disorders includes immunotherapy such as corticosteroids, intravenous immunoglobulin or plasma exchange. Improvements in clinical criteria and the development of more disease-specific immunotherapies should expand the therapeutic possibilities for these debilitating diseases.
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Affiliation(s)
- Edyta Dziadkowiak
- Department of Neurology, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland
| | - Marta Nowakowska-Kotas
- Department of Neurology, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland
| | - Wiktoria Rałowska-Gmoch
- Department of Neurology, The St. Jadwiga's Regional Specialist Neuropsychiatric Centre, Wodociągowa 4, 45-221 Opole, Poland
| | - Sławomir Budrewicz
- Department of Neurology, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland
| | - Magdalena Koszewicz
- Department of Neurology, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland
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21
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Nakamura Y, Itoh Y, Wakimoto N, Kanno R, Iida S, Yamamoto K. Successful treatment with bortezomib for POEMS syndrome, overcoming complicated severe heart block. Clin Case Rep 2023; 11:e7004. [PMID: 36873076 PMCID: PMC9979967 DOI: 10.1002/ccr3.7004] [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: 11/14/2022] [Revised: 01/10/2023] [Accepted: 02/06/2023] [Indexed: 03/06/2023] Open
Abstract
Cardiac bradyarrhythmia and conduction disorder may be rare, but recurrent adverse events caused by bortezomib. Here we report a case with POEMS syndrome presenting severe heart block after bortezomib plus dexamethasone therapy. After permanent pacemaker implantation, bortezomib was restarted and maintained, resulting in sustained complete response for POEMS syndrome.
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Affiliation(s)
- Yuichi Nakamura
- Department of HematologySaitama Medical University HospitalMoroyamaJapan
| | - Yoshihiro Itoh
- Department of HematologySaitama Medical University HospitalMoroyamaJapan
| | - Naoki Wakimoto
- Department of HematologySaitama Medical University HospitalMoroyamaJapan
| | - Ryu Kanno
- Department of Cardiovascular MedicineSaitama Medical University HospitalMoroyamaJapan
| | - Shinichirou Iida
- Department of Cardiovascular MedicineSaitama Medical University HospitalMoroyamaJapan
| | - Keiji Yamamoto
- Department of Cardiovascular MedicineSaitama Medical University HospitalMoroyamaJapan
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22
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Zhang YH, He YF, Yue H, Zhang YN, Shi L, Jin B, Dong P. Solitary hyoid plasmacytoma with unicentric Castleman disease: A case report and review of literature. World J Clin Cases 2022; 10:13364-13372. [PMID: 36683640 PMCID: PMC9850993 DOI: 10.12998/wjcc.v10.i36.13364] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/16/2022] [Revised: 09/17/2022] [Accepted: 12/05/2022] [Indexed: 12/26/2022] Open
Abstract
BACKGROUND Solitary plasmacytoma and unicentric Castleman disease (UCD) are rare lymphoproliferative disorders characterized by monoclonal plasma cells and a single set of locally enlarged lymph nodes, respectively.
CASE SUMMARY A 48-year-old Han Chinese man presented to our department with a neck mass and progressive foreign body sensation in his throat. 18F-FDG positron emission tomography revealed focally increased radioactivity centered around the hyoid, and computed tomography (CT) revealed osteolytic lesions. Histopathology revealed Castleman-like features and CD138/CD38-positive mature plasma cells. Systemic work-up ruled out the possibility of POEMS syndrome, lymphoma, and multiple myeloma, leading to a final diagnosis of solitary hyoid plasmacytoma with UCD. The patient underwent partial hyoid resection and selective neck dissection, followed by intensity-modulated radiotherapy. 99mTc-MDP single-photon emission computed tomography/CT reevaluation showed neither local recurrence nor distant bone metastasis at the 40-mo follow-up.
CONCLUSION The diagnostic process and differential diagnosis of this rare case provided valuable educational information to clinicians.
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Affiliation(s)
- Yan-Hui Zhang
- Department of Otolaryngology-Head and Neck Surgery, Shanghai General Hospital, Shanghai Jiao Tong University, Shanghai 200080, China
| | - Yi-Feng He
- Department of Radiology, Shanghai General Hospital, Shanghai Jiao Tong University, Shanghai 200080, China
| | - Hao Yue
- Department of Pathology, Shanghai Cancer Center, Fudan University, Shanghai 200032, China
| | - Yue-Ni Zhang
- NHC Key Laboratory of Molecular Probes and Targeted Diagnosis and Therapy, The Fourth Affiliated Hospital of Harbin Medical University, Harbin 150028, Heilongjiang Province, China
| | - Lei Shi
- NHC Key Laboratory of Molecular Probes and Targeted Diagnosis and Therapy, The Fourth Affiliated Hospital of Harbin Medical University, Harbin 150028, Heilongjiang Province, China
| | - Bin Jin
- Department of Otolaryngology-Head and Neck Surgery, Shanghai General Hospital, Shanghai Jiao Tong University, Shanghai 200080, China
| | - Pin Dong
- Department of Otolaryngology-Head and Neck Surgery, Shanghai General Hospital, Shanghai Jiao Tong University, Shanghai 200080, China
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23
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McCombe PA, Hardy TA, Nona RJ, Greer JM. Sex differences in Guillain Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and experimental autoimmune neuritis. Front Immunol 2022; 13:1038411. [PMID: 36569912 PMCID: PMC9780466 DOI: 10.3389/fimmu.2022.1038411] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2022] [Accepted: 11/24/2022] [Indexed: 12/13/2022] Open
Abstract
Guillain Barré syndrome (GBS) and its variants, and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP and its variants, are regarded as immune mediated neuropathies. Unlike in many autoimmune disorders, GBS and CIDP are more common in males than females. Sex is not a clear predictor of outcome. Experimental autoimmune neuritis (EAN) is an animal model of these diseases, but there are no studies of the effects of sex in EAN. The pathogenesis of GBS and CIDP involves immune response to non-protein antigens, antigen presentation through non-conventional T cells and, in CIDP with nodopathy, IgG4 antibody responses to antigens. There are some reported sex differences in some of these elements of the immune system and we speculate that these sex differences could contribute to the male predominance of these diseases, and suggest that sex differences in peripheral nerves is a topic worthy of further study.
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Affiliation(s)
- Pamela A. McCombe
- Centre for Clinical Research, The University of Queensland, Brisbane, QLD, Australia
| | - Todd A. Hardy
- Department of Neurology, Concord Hospital, University of Sydney, Sydney, NSW, Australia
- Brain & Mind Centre, University of Sydney, Sydney, NSW, Australia
| | - Robert J. Nona
- Centre for Clinical Research, The University of Queensland, Brisbane, QLD, Australia
| | - Judith M. Greer
- Centre for Clinical Research, The University of Queensland, Brisbane, QLD, Australia
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24
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Ríos-Tamayo R, Paiva B, Lahuerta JJ, López JM, Duarte RF. Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal. Cancers (Basel) 2022; 14:5247. [PMID: 36358666 PMCID: PMC9659226 DOI: 10.3390/cancers14215247] [Citation(s) in RCA: 10] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2022] [Revised: 10/15/2022] [Accepted: 10/24/2022] [Indexed: 11/26/2022] Open
Abstract
Monoclonal gammopathies of clinical significance (MGCSs) represent a group of diseases featuring the association of a nonmalignant B cells or plasma cells clone, the production of an M-protein, and singularly, the existence of organ damage. They present a current framework that is difficult to approach from a practical clinical perspective. Several points should be addressed in order to move further toward a better understanding. Overall, these entities are only partially included in the international classifications of diseases. Its definition and classification remain ambiguous. Remarkably, its real incidence is unknown, provided that a diagnostic biopsy is mandatory in most cases. In fact, amyloidosis AL is the final diagnosis in a large percentage of patients with renal significance. On the other hand, many of these young entities are syndromes that are based on a dynamic set of diagnostic criteria, challenging a timely diagnosis. Moreover, a specific risk score for progression is lacking. Despite the key role of the clinical laboratory in the diagnosis and prognosis of these patients, information about laboratory biomarkers is limited. Besides, the evidence accumulated for many of these entities is scarce. Hence, national and international registries are stimulated. In particular, IgM MGCS deserves special attention. Until now, therapy is far from being standardized, and it should be planned on a risk and patient-adapted basis. Finally, a comprehensive and coordinated multidisciplinary approach is needed, and specific clinical trials are encouraged.
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Affiliation(s)
- Rafael Ríos-Tamayo
- Hospital Universitario Puerta de Hierro, Fundación para la Investigación Biomédica del Hospital Universitario Puerta de Hierro-Majadahonda, 28222 Majadahonda, Spain
| | - Bruno Paiva
- Centro de Investigación Médica Aplicada, Instituto de Investigación Sanitaria de Navarra, Clínica Universidad de Navarra, 31008 Pamplona, Spain
| | - Juan José Lahuerta
- Hospital Universitario 12 de Octubre, Instituto de Investigación del Hospital Universitario 12 de Octubre, 28041 Madrid, Spain
| | - Joaquín Martínez López
- Hospital Universitario 12 de Octubre, Instituto de Investigación del Hospital Universitario 12 de Octubre, 28041 Madrid, Spain
| | - Rafael F. Duarte
- Hospital Universitario Puerta de Hierro, Fundación para la Investigación Biomédica del Hospital Universitario Puerta de Hierro-Majadahonda, 28222 Majadahonda, Spain
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25
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Kang L, Wan C. Paraneoplastic syndrome in neuroophthalmology. J Neurol 2022; 269:5272-5282. [PMID: 35779086 DOI: 10.1007/s00415-022-11247-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2022] [Revised: 06/19/2022] [Accepted: 06/19/2022] [Indexed: 12/16/2022]
Abstract
Paraneoplastic syndrome is a group of clinical symptoms that occur in the state of systemic malignant tumors. Paraneoplastic syndrome of the nervous system can affect any part of the central and peripheral nervous system and may also affect the eyes. In neuroophthalmology, paraneoplastic syndrome has a variety of manifestations that can affect both the afferent and efferent visual systems. The afferent system may involve the optic nerve, retina and uvea; the efferent system may involve eye movement, neuromuscular joints or involuntary eye movements and pupil abnormalities and may also have other neurological symptoms outside the visual system. This article discusses the clinical manifestations, pathological mechanisms, detection methods and treatment methods of paraneoplastic syndrome in neuroophthalmology. The performance of paraneoplastic syndrome is diverse, the diagnosis is difficult, and the treatment should be considered systematically. Differential diagnosis, optimal evaluation and management of these manifestations is not only the key to treatment but also a challenge.
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Affiliation(s)
- Longdan Kang
- Department of Ophthalmology, The First Hospital of China Medical University, 155 Nanjing North Street, Heping District, Shenyang, 110001, China
| | - Chao Wan
- Department of Ophthalmology, The First Hospital of China Medical University, 155 Nanjing North Street, Heping District, Shenyang, 110001, China.
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26
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Outcomes after autologous hematopoietic cell transplantation in POEMS syndrome and comparison with multiple myeloma. Blood Adv 2022; 6:3991-3995. [PMID: 35507742 PMCID: PMC9278304 DOI: 10.1182/bloodadvances.2022007218] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2022] [Accepted: 04/27/2022] [Indexed: 11/20/2022] Open
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27
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Zhou T, Wang HW, Pittaluga S, Jaffe ES. Multicentric Castleman disease and the evolution of the concept. Pathologica 2021; 113:339-353. [PMID: 34837092 PMCID: PMC8720411 DOI: 10.32074/1591-951x-351] [Citation(s) in RCA: 17] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2021] [Accepted: 08/25/2021] [Indexed: 12/29/2022] Open
Abstract
The term multicentric Castleman disease (MCD) encompasses a spectrum of conditions that share some overlapping clinicopathological manifestations. The fundamental pathogenetic mechanism involves dysregulated cytokine activity, causing systemic inflammatory symptoms as well as lymphadenopathy. Some of the histological changes in lymph nodes resemble the histology of unicentric Castleman disease (UCD). However, based on current knowledge, the use of this shared nomenclature is unfortunate, since these disorders differ in pathogenesis and prognosis. In Kaposi sarcoma-associated herpesvirus (KSHV)-associated MCD, cytokine overactivity is caused by viral products, which can also lead to atypical lymphoproliferations and potential progression to lymphoma. In idiopathic MCD, the hypercytokinemia can result from various mechanisms, which ultimately lead to different constellations of clinical presentations and varied pathology in lymphoid tissues. The authors review the evolving concepts and definitions of the various conditions under the eponym of multicentric Castleman disease.
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Affiliation(s)
- Ting Zhou
- Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
| | - Hao-Wei Wang
- Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
| | - Stefania Pittaluga
- Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
| | - Elaine S Jaffe
- Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
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