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Dotlic S, Gibson SE, Hartmann S, Hsi ED, Klimkowska M, Rodriguez-Pinilla SM, Sabattini E, Tousseyn TA, de Jong D, Dojcinov S. Lymphomas with plasmablastic features: a report of the lymphoma workshop of the 20th meeting of the European Association for Haematopathology. Virchows Arch 2023; 483:591-609. [PMID: 37561194 DOI: 10.1007/s00428-023-03585-8] [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: 03/20/2023] [Revised: 06/16/2023] [Accepted: 06/22/2023] [Indexed: 08/11/2023]
Abstract
Lymphomas with plasmablastic features are a heterogeneous group of aggressive and mostly uncommon neoplasms of varied aetiologies, presenting in immunocompetent individuals as well as in immunodeficiency, associated with EBV and Kaposi sarcoma virus infections, and some as progression from indolent B-cell lymphomas. They show overlapping diagnostic features and pose a differential diagnosis with other aggressive B-cell lymphomas that can downregulate the B-cell expression programme. The spectrum of rare reactive proliferations and all lymphomas defined by plasmablastic features, together with an expanding range of poorly characterised, uncommon conditions at the interface between reactive lymphoid proliferations and neoplasia submitted to the session V of the 20th European Association for Haematopathology/Society for Hematopathology lymphoma workshop are summarised and discussed in this paper.
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Affiliation(s)
- Snjezana Dotlic
- Department of Pathology and Cytology, University Hospital Centre Zagreb, University of Zagreb Medical School, Zagreb, Croatia
| | - Sarah E Gibson
- Division of Hematopathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Phoenix, AZ, USA
| | - Sylvia Hartmann
- Dr. Senckenberg Institute of Pathology, Goethe University Frankfurt Am Main, Frankfurt Am Main, Germany
| | - Eric D Hsi
- Department of Pathology, Wake Forest University School of Medicine, Winston-Salem, USA
| | - Monika Klimkowska
- Department of Clinical Pathology and Cancer Diagnostics, Karolinska University Hospital Huddinge, Stockholm, Sweden
| | | | - Elena Sabattini
- Haematopathology Unit, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Bologna, Italy
| | - Thomas A Tousseyn
- Department of Imaging and Pathology and Translational Cell and Tissue Research Laboratory, Louvain, Belgium
| | - Daphne de Jong
- Department of Pathology, Amsterdam UMC, Location VUMC, Amsterdam, The Netherlands
| | - Stefan Dojcinov
- Department of Pathology, Morriston Hospital, Swansea Bay University Health Board/Swansea University, Swansea, UK.
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2
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Mwazha A, Nhlonzi GB, Mazengenya P. Gastrointestinal Tract Plasmablastic Lymphoma in HIV-Infected Adults: A Histopathological Review. Int J Surg Pathol 2020; 28:735-748. [PMID: 32552168 DOI: 10.1177/1066896920932272] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
BACKGROUND. Plasmablastic lymphoma (PBL) is an aggressive B-cell lymphoma that is characterized by the expression of plasma cell antigens and loss of pan B-cell antigens. The neoplasm is extensively reported in the oral cavity and anorectal region but rarely in the gastrointestinal tract, where only isolated case reports and small case series have been reported. In the current study, morphologic, immunohistochemical, and molecular features of 17 cases of gastrointestinal tract PBL were reviewed. METHODS. Ten-year retrospective study that reappraised the histomorphological and immunophenotypical profiles of HIV-associated PBLs in the gastrointestinal tract. RESULTS. The mean age of the study patients was 41 years with a 3:1 ratio of males to females. The most common site of tumor origin was the small intestine (42%), followed by the stomach (29%) and the colon (29%). Majority of the cases showed a predominant diffuse (82%) growth pattern. Immunoblasts and plasmablasts were observed in all cases. Plasmacytic differentiation was seen in 5 (29%) cases. Additional observations not previously described or emphasized in literature includes pseudo-alveolar growth pattern, centroblast-predominance, multinucleated giant cells, and clear cell change. Immunohistochemistry revealed absence of pan B-cell antigens and expression of plasma cell antigens in all cases. Epstein-Barr virus-encoded RNA was expressed in 53% of the cases. CONCLUSIONS. This study highlights the spectrum of histopathological features seen in gastrointestinal tract PBLs. Awareness of this entity and its histopathological features in the gastrointestinal tract is essential for making a timely and accurate diagnosis and improving patient outcomes.
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Affiliation(s)
- Absalom Mwazha
- University of KwaZulu-Natal, Durban, South Africa.,National Health Laboratory Services, Durban, South Africa
| | | | - Pedzisai Mazengenya
- University of the Witwatersrand, Johannesburg, South Africa.,Ajman University, Ajman, United Arab Emirates
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3
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Abstract
The contribution of Epstein-Barr virus (EBV) to the development of specific types of benign lymphoproliferations and malignant lymphomas has been extensively studied since the discovery of the virus over the last 50 years. The importance and better understanding of the EBV-associated lymphoproliferative disorders (LPD) of B, T or natural killer (NK) cell type has resulted in the recognition of new entities like EBV+ mucocutaneous ulcer or the addition of chronic active EBV (CAEBV) infection in the revised 2016 World Health Organization (WHO) lymphoma classification. In this article, we review the definitions, morphology, pathogenesis, and evolving concepts of the various EBV-associated disorders including EBV+ diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS), EBV+ mucocutaneous ulcer, DLBCL associated with chronic inflammation, fibrin-associated DLBCL, lymphomatoid granulomatosis, the EBV+ T and NK-cell LPD of childhood, aggressive NK leukaemia, extranodal NK/T-cell lymphoma, nasal type, and the new provisional entity of primary EBV+ nodal T- or NK-cell lymphoma. The current knowledge regarding the pathogenesis of B-cell lymphomas that can be EBV-associated including Burkitt lymphoma, plasmablastic lymphoma and classic Hodgkin lymphoma will be also explored.
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Linke-Serinsöz E, Fend F, Quintanilla-Martinez L. Human immunodeficiency virus (HIV) and Epstein-Barr virus (EBV) related lymphomas, pathology view point. Semin Diagn Pathol 2017; 34:352-363. [PMID: 28506687 DOI: 10.1053/j.semdp.2017.04.003] [Citation(s) in RCA: 47] [Impact Index Per Article: 5.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
The contribution of Epstein Barr virus (EBV) and Kaposi sarcoma herpes virus (KSHV) to the development of specific types of malignant lymphomas occurring in the human immunodeficiency virus (HIV) setting has been extensively studied since the beginning of the HIV epidemic 35 years ago. The introduction of highly active antiretroviral therapies (HAART) in 1996 has changed dramatically the incidence of HIV-related malignancies. Nevertheless, malignant lymphomas continue to be the major group of malignances observed in HIV infected individuals, and the most common cause of cancer related-deaths. Common features of the predominant B-cell lymphomas in the HIV+ setting are the frequent plasmacytoid morphology of the neoplastic cells, advanced stage, aggressive disease and frequent extranodal involvement. In this article, we review the evolving concepts and definitions of the various EBV-associated lymphomas in HIV+ patients, including diffuse large B-cell lymphoma, Burkitt lymphoma, classical Hodgkin lymphoma, plasmablastic lymphoma and primary effusion lymphoma. The current knowledge regarding the pathogenesis of these malignancies, the interplay between HIV and EBV co-infection in the development of certain HIV related lymphomas, and the emerging paradigm that suggests that HIV may play a direct role in lymphomagenesis are explored as well.
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Affiliation(s)
- Ebru Linke-Serinsöz
- Institute of Pathology, University Hospital Tübingen, Eberhard-Karls-University of Tübingen and Comprehensive Cancer Center, Tübingen, Germany
| | - Falko Fend
- Institute of Pathology, University Hospital Tübingen, Eberhard-Karls-University of Tübingen and Comprehensive Cancer Center, Tübingen, Germany
| | - Leticia Quintanilla-Martinez
- Institute of Pathology, University Hospital Tübingen, Eberhard-Karls-University of Tübingen and Comprehensive Cancer Center, Tübingen, Germany.
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Mani SSR, Kodiatte T, Jagannati M. A rare presentation of plasmablastic lymphoma as cutaneous nodules in an immunocompromised patient. Int J STD AIDS 2016; 28:623-625. [PMID: 27738277 DOI: 10.1177/0956462416675037] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Plasmablastic lymphoma is a rare entity accounting for around 2.7% of all AIDS-related lymphomas. The oral cavity and gastrointestinal tract are the most common sites involved. We report a case of a 34-year-old HIV-positive woman with a rare presentation of cutaneous nodules all over the body. Due to overwhelming tumour burden, she developed tumour lysis syndrome during her hospital stay and succumbed to the illness.
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Affiliation(s)
| | - Thomas Kodiatte
- 2 Department of General Pathology, Christian Medical College, Vellore, India
| | - Manjeera Jagannati
- 1 Department of General Medicine, Christian Medical College, Vellore, India
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Fernández-Álvarez R, Sancho JM, Ribera JM. [Plasmablastic lymphoma]. Med Clin (Barc) 2016; 147:399-404. [PMID: 27576534 DOI: 10.1016/j.medcli.2016.06.036] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2016] [Revised: 06/11/2016] [Accepted: 06/13/2016] [Indexed: 02/01/2023]
Abstract
Plasmablastic lymphoma (PBL) is a rare and aggressive subtype of non-Hodgkin lymphoma that commonly occurs in human immunodeficiency virus (HIV)-positive individuals, and affects oral sites. Occasionally, it has been described in HIV-negative patients and involving non-oral sites. Pathologically, PBL is a high-grade B-cell lymphoma that displays the immunophenotype of a terminally differentiated B-lymphocyte with loss of B-cell markers (CD20) and expression of plasma-cell antigens. Epstein-Barr virus infection and MYC rearrangements are frequently observed. Treatment of PBL is challenging because of the lack of established treatment and poor outcomes, with median survival times shorter than one year. In this review, we discuss the clinical and epidemiologic spectrum of PBL as well as its distinct pathological features. Finally, we summarize the currently available approaches for the treatment of patients with PBL.
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Affiliation(s)
| | - Juan-Manuel Sancho
- Servicio de Hematología Clínica, Institut Català d'Oncologia, Hospital Germans Trias i Pujol, Institut de Recerca contra la Leucèmia Josep Carreras, Universitat Autònoma de Barcelona, Badalona, Barcelona, España
| | - Josep-María Ribera
- Servicio de Hematología Clínica, Institut Català d'Oncologia, Hospital Germans Trias i Pujol, Institut de Recerca contra la Leucèmia Josep Carreras, Universitat Autònoma de Barcelona, Badalona, Barcelona, España
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7
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Laurent C, Fabiani B, Do C, Tchernonog E, Cartron G, Gravelle P, Amara N, Malot S, Palisoc MM, Copie-Bergman C, Glehen AT, Copin MC, Brousset P, Pittaluga S, Jaffe ES, Coppo P. Immune-checkpoint expression in Epstein-Barr virus positive and negative plasmablastic lymphoma: a clinical and pathological study in 82 patients. Haematologica 2016; 101:976-84. [PMID: 27175027 PMCID: PMC4967577 DOI: 10.3324/haematol.2016.141978] [Citation(s) in RCA: 64] [Impact Index Per Article: 7.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/27/2016] [Accepted: 05/03/2016] [Indexed: 12/14/2022] Open
Abstract
Plasmablastic lymphoma is a rare and aggressive diffuse large B-cell lymphoma commonly associated with Epstein-Barr virus co-infection that most often occurs in the context of human immunodeficiency virus infection. Therefore, its immune escape strategy may involve the upregulation of immune-checkpoint proteins allowing the tumor immune evasion. However, the expression of these molecules was poorly studied in this lymphoma. We have investigated 82 plasmablastic lymphoma cases of whom half were Epstein-Barr virus positive. Although they harbored similar pathological features, Epstein-Barr virus positive plasmablastic lymphomas showed a significant increase in MYC gene rearrangement and had a better 2-year event-free survival than Epstein-Barr virus negative cases (P=0.049). Immunostains for programmed cell death-1, programmed cell death-ligand 1, indole 2,3-dioxygenase and dendritic cell specific C-type lectin showed a high or moderate expression by the microenvironment cells in 60%-72% of cases, whereas CD163 was expressed in almost all cases. Tumor cells also expressed programmed cell death-1 and its ligand in 22.5% and 5% of cases, respectively. Both Epstein-Barr virus positive and negative plasmablastic lymphomas exhibited a high immune-checkpoint score showing that it involves several pathways of immune escape. However, Epstein-Barr virus positive lymphomas exhibited a higher expression of programmed cell death-1 and its ligand in both malignant cells and microenvironment as compared to Epstein-Barr virus negative cases. In conclusion, plasmablastic lymphoma expresses immune-checkpoint proteins through both malignant cells and the tumor microenvironment. The expression of programmed cell death-1 and its ligand constitutes a strong rationale for testing monoclonal antibodies in this often chemoresistant disease.
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Affiliation(s)
- Camille Laurent
- Département de Pathologie, Institut Universitaire du Cancer-Oncopole, Toulouse, France INSERM, U.1037, Centre de Recherche en Cancérologie de Toulouse-Purpan, Toulouse, France
| | - Bettina Fabiani
- Département de Pathologie, AP-HP, Hôpital Saint-Antoine, Paris, France
| | - Catherine Do
- Institute for Cancer Genetics, Columbia University, New York, NY, USA
| | | | - Guillaume Cartron
- Service d'Hematologie, Hôpital Gui de Chauliac-Saint Eloi, Montpellier, France
| | - Pauline Gravelle
- Département de Pathologie, Institut Universitaire du Cancer-Oncopole, Toulouse, France INSERM, U.1037, Centre de Recherche en Cancérologie de Toulouse-Purpan, Toulouse, France
| | - Nadia Amara
- Département de Pathologie, Institut Universitaire du Cancer-Oncopole, Toulouse, France
| | - Sandrine Malot
- Service d'Hématologie, AP-HP, Hôpital Saint-Antoine, Paris, France Centre de Référence des Microangiopathies Thrombotiques, AP-HP, Paris, France
| | | | - Christiane Copie-Bergman
- Département de Pathologie, AP-HP, Groupe Hospitalier Henri Mondor - Albert henevier, Créteil, France
| | | | | | - Pierre Brousset
- Département de Pathologie, Institut Universitaire du Cancer-Oncopole, Toulouse, France INSERM, U.1037, Centre de Recherche en Cancérologie de Toulouse-Purpan, Toulouse, France
| | - Stefania Pittaluga
- Hematopathology Section, Laboratory of Pathology, National Cancer Institute, Bethesda, MD, USA
| | - Elaine S Jaffe
- Hematopathology Section, Laboratory of Pathology, National Cancer Institute, Bethesda, MD, USA
| | - Paul Coppo
- Service d'Hématologie, AP-HP, Hôpital Saint-Antoine, Paris, France Centre de Référence des Microangiopathies Thrombotiques, AP-HP, Paris, France UPMC, Université Paris VI, France Inserm U1170, Institut Gustave Roussy, Villejuif, France
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8
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Wang B, Song B, Oster C, Cao J, Raza A, Wang J. Coexistence of intestinal Kaposi sarcoma and plasmablastic lymphoma in an HIV/AIDS patient: case report and review of the literature. J Gastrointest Oncol 2016; 7:S88-95. [PMID: 27034819 PMCID: PMC4783623 DOI: 10.3978/j.issn.2078-6891.2015.021] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/16/2015] [Accepted: 01/26/2015] [Indexed: 12/19/2022] Open
Abstract
Human immunodeficiency virus (HIV) infection or acquired immunodeficiency disease (AIDS) is associated with increased risk for various malignancies including Kaposi sarcoma (KS) and lymphoma. We report a rare case of coexistence of KS and plasmablastic lymphoma (PBL) in the gastrointestinal (GI) tract in a HIV/AIDS patient. A brief review of literature is also presented.
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Affiliation(s)
- Bing Wang
- Department of Pathology, Loma Linda University Medical Center, CA 92354, USA
| | - Bingbing Song
- Department of Pathology, Loma Linda University Medical Center, CA 92354, USA
| | - Cyrus Oster
- Department of Pathology, Loma Linda University Medical Center, CA 92354, USA
| | - Jeffery Cao
- Department of Pathology, Loma Linda University Medical Center, CA 92354, USA
| | - Anwar Raza
- Department of Pathology, Loma Linda University Medical Center, CA 92354, USA
| | - Jun Wang
- Department of Pathology, Loma Linda University Medical Center, CA 92354, USA
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Clinical pathologic conference case 2: gingival ulcer in a 34-year-old man. Oral Surg Oral Med Oral Pathol Oral Radiol 2016; 119:e274-9. [PMID: 26046140 DOI: 10.1016/j.oooo.2014.11.005] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
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Plasmablastic Lymphoma: A Review of Current Knowledge and Future Directions. Adv Hematol 2015; 2015:315289. [PMID: 26357515 PMCID: PMC4555447 DOI: 10.1155/2015/315289] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/16/2015] [Revised: 07/29/2015] [Accepted: 08/03/2015] [Indexed: 12/16/2022] Open
Abstract
Plasmablastic lymphoma (PBL) is an aggressive subtype of non-Hodgkin's lymphoma (NHL), which frequently arises in the oral cavity of human immunodeficiency virus (HIV) infected patients. PBL shows diffuse proliferation of large neoplastic cells resembling B-immunoblasts/plasmablasts, or with plasmacytic features and an immunophenotype of plasma cells. PBL remains a diagnostic challenge due to its peculiar morphology and an immunohistochemical profile similar to plasma cell myeloma (PCM). PBL is also a therapeutic challenge with a clinical course characterized by a high rate of relapse and death. There is no standard chemotherapy protocol for treatment of PBL. Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or CHOP-like regimens have been the backbone while more intensive regimens such as cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate/ifosfamide, etoposide, high-dose cytarabine (CODOX-M/IVAC), or dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-EPOCH) are possible options. Recently, a few studies have reported the potential value of the proteasome inhibitor bortezomib and thalidomide in PBL patients. The introduction of genes encoding artificial receptors called chimeric antigen receptors (CARs) and CAR-modified T cells targeted to the B cell-specific CD19 antigen have demonstrated promising results in multiple early clinical trials. The aim of this paper is to review the recent advances in epidemiology; pathophysiology; clinical, pathologic, and molecular characteristics; therapy; and outcome in patients with PBL.
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11
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Clinicopathologic features of plasmablastic lymphoma: Single-center series of 8 cases from Saudi Arabia. Diagn Pathol 2015; 10:78. [PMID: 26108914 PMCID: PMC4479229 DOI: 10.1186/s13000-015-0315-z] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2015] [Accepted: 06/03/2015] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Plasmablastic lymphoma (PBL) is a rare subtype of non-Hodgkin's lymphoma. Characterized by its aggressive nature and plasmacytic differentiation, PBL remains a therapeutic and diagnostic challenge; it generally has a poor prognosis with very few long-term survivors and most patients dying within 2 years from initial presentation. PBL has been reported in several other countries; however, there have been no reported cases from Saudi Arabia. Here, we report 8 cases of PBL depicting the clinical presentation, immunocompetency, immunphenotypic characterization, diagnostic challenges and treatment outcome. METHODS The medical records were reviewed for clinical presentation, staging, laboratory data, radiological studies, treatments, and outcomes. A broad immunohistochemical panel consisting of CD45, CD3, CD20, CD79a, Pax5, CD38, CD138, MUM1, EMA, Kappa, Lambda, CD 56, CD30, Bcl-2, Bcl-6, Alk-1, Ki-67, EBV-LMP-1, and HHV8 was performed. RESULTS The tumors predominantly exhibited immunoblastic/plasmablastic or plasmacytic morphologic features and had a plasma cell-like immunophenotype. All cases were immunoreactive for CD38, CD138 and MUM1 confirming plasma cell differentiation of the tumor cells. CD20 was negative for all cases; whereas CD79a and Pax5 were weakly positive in 2cases. All 8 cases were EBV-LMP-1/EBER-1 negative, and 1 case was HHV8 positive. Similar to previously published studies, PBL in Saudi Arabia is characterized by male predominance (6/8), median age 51.5 years (mean age 46 years), associated with early dissemination, poor response to therapy, and limited survival (average survival time, 6.4 months, median overall survival 5.5 months). However, it does have some unique features. It occurs more commonly in immunocompetent persons (6/8, 75%), is not associated with EBV infection (0/8), and nodal involvement (either primary or secondary) is common among patients (6/8). In addition, extra-oral sites are more common than oral/nasal cavities (7/8) and the c-myc gene is not common (1/8, 12.5%). CONCLUSION It appears that PBL is heterogeneous in terms of clinical presentation and morphology. PBL is a therapeutic challenge with a clinical course that is characterized by its high rate of relapse and death. To date, treatment responses are usually partial and temporary. Therapies that are more intensive than CHOP do not seem to prolong survival. Further research is needed to understand the biology and molecular pathogenesis of PBL in order to improve therapies. VIRTUAL SLIDES The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1465801416161912.
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Chagas LA, Camilo GB, Machado DC, Vidal DR, de Oliveira CE, Toledo GC, Oria GPC, Silva MDF, Oliveira RVD, Lopes AJ. Plasmablastic lymphoma of the anal canal in an HIV-infected patient. AMERICAN JOURNAL OF CASE REPORTS 2014; 15:543-9. [PMID: 25479715 PMCID: PMC4260690 DOI: 10.12659/ajcr.892313] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
BACKGROUND The advent of antiretroviral therapy increased the life expectancy of human immunodeficiency virus (HIV)-positive patients and, consequently, the morbidity and mortality due to neoplasms. Plasmablastic lymphoma is one such neoplasm that generally presents with involvement of the oral cavity; cases of extra-oral involvement are rare. CASE REPORT We report a case of plasmablastic lymphoma in a 46-year-old woman for whom the initial clinical manifestation was a painless perineal tumor accompanied by fecal incontinence. CONCLUSIONS The possibility of this neoplasm should be considered in patients with HIV/acquired immune deficiency syndrome (HIV/AIDS) because its early diagnosis is essential so that the start of the treatment is not delayed.
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Affiliation(s)
- Lucia Antunes Chagas
- Department of Radiology, State University of Rio de Janeiro, Rio de Janeiro, Brazil
| | | | | | - Débora Ribeiro Vidal
- Department of Clinical Medicine, State University of Rio de Janeiro, Rio de Janeiro, Brazil
| | | | - Gabriela Cumani Toledo
- Therezinha de Jesus Hospital and Maternity, Faculty of Medical and Health Sciences of Juiz de Fora, Juiz de Fora, Brazil
| | | | - Monique de França Silva
- Department of Anatomic Pathology, State University of Rio de Janeiro, Rio de Janeiro, Brazil
| | | | - Agnaldo José Lopes
- Postgraduate Programe in Medical Sciences, State University of Rio de Janeiro, Rio de Janeiro, Brazil
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13
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Harris E, Butler JS, Cassidy N. Aggressive plasmablastic lymphoma of the thoracic spine presenting as acute spinal cord compression in a case of asymptomatic undiagnosed human immunodeficiency virus infection. Spine J 2014; 14:e1-5. [PMID: 24362000 DOI: 10.1016/j.spinee.2013.12.018] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/06/2012] [Revised: 10/28/2013] [Accepted: 12/14/2013] [Indexed: 02/03/2023]
Abstract
BACKGROUND CONTEXT Plasmablastic lymphoma (PBL) is a rare aggressive variant of diffuse large B-cell lymphoma. PURPOSE We describe a rare case of an aggressive PBL presenting as acute spinal cord compression requiring thoracic decompression and fusion, in a case of previously undiagnosed human immunodeficiency virus (HIV) infection. STUDY DESIGN A case report. PATIENT SAMPLE A patient with PBL of the thoracic spine. OUTCOME MEASURES Preoperative magnetic resonance imaging, pathologic findings from the operative specimen, and serum HIV testing confirmed the diagnosis. METHODS We present the case of a 33-year-old Caucasian woman with a 10-day history of thoracic back pain and a 1-day history of sudden-onset bilateral lower limb weakness and paresthesia from below the level of the umbilicus (American Spinal Injury Association [ASIA] Grade C). Magnetic resonance imaging demonstrated an extradural mass extending from T3 to T6 within the left posterior canal, resulting in significant cord compression. A complete debulking of the tumor mass and an instrumented posterior thoracic fusion was performed. RESULTS Histopathologic examination of the specimen revealed tumor cells of PBL, and subsequent HIV testing was positive. She was treated with intravenous and intrathecal chemotherapy to prevent recurrence. Her lower limb neurologic status improved to ASIA Grade D over the subsequent 2 weeks. CONCLUSIONS We report the case of an aggressive PBL presenting as acute spinal cord compression requiring urgent surgical intervention, on a background of undiagnosed HIV infection.
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Affiliation(s)
- Ella Harris
- National Spinal Injuries Unit, Department of Trauma & Orthopaedic Surgery, Mater Misericordiae University Hospital, Eccles St, Dublin 7, Ireland.
| | - Joseph S Butler
- National Spinal Injuries Unit, Department of Trauma & Orthopaedic Surgery, Mater Misericordiae University Hospital, Eccles St, Dublin 7, Ireland
| | - Noelle Cassidy
- National Spinal Injuries Unit, Department of Trauma & Orthopaedic Surgery, Mater Misericordiae University Hospital, Eccles St, Dublin 7, Ireland
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14
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Tani J, Miyoshi H, Nomura T, Yoneyama H, Kobara H, Mori H, Morishita A, Himoto T, Masaki T. A case of plasmablastic lymphoma of the liver without human immunodeficiency virus infection. World J Gastroenterol 2013; 19:6299-6303. [PMID: 24115831 PMCID: PMC3787364 DOI: 10.3748/wjg.v19.i37.6299] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/03/2013] [Revised: 08/07/2013] [Accepted: 08/20/2013] [Indexed: 02/06/2023] Open
Abstract
Plasmablastic lymphoma (PBL) is a very rare B-cell lymphoproliferative disorder was with an aggressive clinical behavior that recently characterized by the World Health Organization. Although PBL is most commonly observed in the oral cavity of human immunodeficiency virus (HIV)-positive patients, it can also be observed at extra-oral sites in HIV-negative patients. Epstein-Barr virus (EBV) may be closely related the pathogenesis of PBL. PBL shows different clinicopathological characteristics between HIV-positive and -negative patients. Here, we report a case of PBL of the liver in a 79-year-old HIV-negative male. The patient died approximately 1.5 mo after examination and autopsy showed that the main lesion was a very large liver mass. Histopathological examination of the excised lesion showed large-cell lymphoma with plasmacytic differentiation diffusely infiltrating the liver and involving the surrounding organs. The neoplastic cells were diffusely positive for CD30, EBV, Bob-1, and CD38. The autopsy findings suggested a diagnosis of PBL. To our knowledge, the present case appears to be the first report of PBL with initial presentation of the liver in a patient without HIV infection.
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MESH Headings
- ADP-ribosyl Cyclase 1/analysis
- Aged
- Autopsy
- Biomarkers, Tumor/analysis
- Fatal Outcome
- Herpesvirus 4, Human/isolation & purification
- Humans
- Immunohistochemistry
- Ki-1 Antigen/analysis
- Liver Neoplasms/chemistry
- Liver Neoplasms/pathology
- Liver Neoplasms/therapy
- Liver Neoplasms/virology
- Lymphoma, Large B-Cell, Diffuse/chemistry
- Lymphoma, Large B-Cell, Diffuse/pathology
- Lymphoma, Large B-Cell, Diffuse/therapy
- Lymphoma, Large B-Cell, Diffuse/virology
- Male
- Membrane Glycoproteins/analysis
- Palliative Care
- Tomography, X-Ray Computed
- Trans-Activators/analysis
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15
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Machado I, Traves V, Cruz J, Llombart B, Navarro S, Llombart-Bosch A. Superficial small round-cell tumors with special reference to the Ewing's sarcoma family of tumors and the spectrum of differential diagnosis. Semin Diagn Pathol 2013; 30:85-94. [DOI: 10.1053/j.semdp.2012.01.007] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
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16
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Mulay K, Ali MJ, Reddy VA, Honavar SG. Orbital plasmablastic lymphoma: a clinico-pathological correlation of a rare disease and review of literature. Clin Ophthalmol 2012; 6:2049-57. [PMID: 23271885 PMCID: PMC3526910 DOI: 10.2147/opth.s38282] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/27/2023] Open
Abstract
Ocular involvement by plasmablastic lymphoma is extremely rare with very few reports in the literature. Its morphological and immunological resemblance to plasma cell myeloma makes it a diagnostic challenge, while its clinical course, which is characterized by recurrence and death, makes therapy a challenge for clinicians. We present three cases of plasmablastic lymphoma, each of which has distinct clinicoradiological features, and we also review the literature on orbital plasmablastic lymphomas.
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Affiliation(s)
- Kaustubh Mulay
- Ocular Pathology Service, LV Prasad Eye Institute, Hyderabad, Andhra Pradesh, India
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17
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Abstract
OBJECTIVE To report two cases of laryngeal plasmablastic lymphoma, a rare and relatively recently described form of non-Hodgkin's lymphoma. It has not previously been described in the larynx, nor associated with upper airway obstruction. CASE REPORTS We describe the clinicopathological features of two such cases in human immunodeficiency virus positive patients, and we discuss their unusual presentations and diagnostic features. CONCLUSION When evaluating a laryngeal tumour, plasmablastic lymphoma and other non-Hodgkin's lymphomata should be considered as differential diagnoses, particularly in the setting of a high prevalence of human immunodeficiency virus infection. Biopsy with detailed histopathological and immunohistochemical evaluation is recommended to ensure correct diagnosis and optimal management.
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18
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Favorable outcome of plasmablastic lymphoma expressing both immunoglobulin light chains arising in an immunocompetent man. J Hematop 2012. [DOI: 10.1007/s12308-012-0150-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/28/2022] Open
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19
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Abstract
The incidence of aggressive lymphoma in the setting of HIV infection is significantly increased relative to the general population. Combination antiretroviral therapy (cART) for HIV has reduced the incidence of these neoplasms and has significantly improved clinical outcome for those who do develop lymphoma and require chemotherapy. With the possible exception of those individuals with the most severe immunocompromise, patients with HIV-associated lymphoma can be treated with the same standard immuno-chemotherapy regimens used in the immunocompetent population with similar expectations for good clinical outcome. Infusional regimens like dose adjusted EPOCH-R appear to be highly effective first-line therapy and for relapsed patients high-dose chemotherapy with autologous stem cell support is well-tolerated and effective. However, it should be recognized that there are unique risks associated with management of lymphoma in this patient population. While opportunistic infections are no longer a significant cause of death, antiretroviral agents used for management of HIV infection may interact with chemotherapeutic agents and other adjunctive therapies making communication between the treating Oncologist and the patient's primary HIV treatment provider of prime importance.
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MESH Headings
- Antineoplastic Agents/administration & dosage
- Antineoplastic Agents/therapeutic use
- Antineoplastic Combined Chemotherapy Protocols/administration & dosage
- Antineoplastic Combined Chemotherapy Protocols/therapeutic use
- Antiretroviral Therapy, Highly Active
- Burkitt Lymphoma/mortality
- Burkitt Lymphoma/pathology
- Burkitt Lymphoma/therapy
- Cyclophosphamide/administration & dosage
- Cyclophosphamide/therapeutic use
- Doxorubicin/administration & dosage
- Doxorubicin/therapeutic use
- Hodgkin Disease/mortality
- Hodgkin Disease/pathology
- Hodgkin Disease/therapy
- Humans
- Lymphoma, AIDS-Related/mortality
- Lymphoma, AIDS-Related/pathology
- Lymphoma, AIDS-Related/therapy
- Lymphoma, Large B-Cell, Diffuse/mortality
- Lymphoma, Large B-Cell, Diffuse/pathology
- Lymphoma, Large B-Cell, Diffuse/therapy
- Peripheral Blood Stem Cell Transplantation
- Prednisone/administration & dosage
- Prednisone/therapeutic use
- Survival Analysis
- Transplantation, Autologous
- Vincristine/administration & dosage
- Vincristine/therapeutic use
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Affiliation(s)
- Lawrence D Kaplan
- Adult Lymphoma Program, University of California, San Francisco, CA 94143, USA.
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20
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Guan B, Zhang X, Ma H, Zhou H, Zhou X. A meta-analysis of highly active anti-retroviral therapy for treatment of plasmablastic lymphoma. Hematol Oncol Stem Cell Ther 2012; 3:7-12. [PMID: 20231808 DOI: 10.1016/s1658-3876(10)50050-5] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022] Open
Abstract
BACKGROUND AND OBJECTIVES Plasmablastic lymphoma is a recently described B-cell derived lymphoma. The prognosis of plasmablastic lymphoma patients is usually poor. We performed a systematic review of the literature on the use of highly active anti-retroviral therapy (HAART) and the prognosis of plasmablastic lymphoma. METHODS A comprehensive search of relevant databases, including Medline, Embase, the Cochrane Controlled Trials Register, the Cochrane Library, and the Science Citation Index yielded ten randomized controlled trials. Trials were divided into two groups according to therapy. The rates of plasmablastic lymphoma were analyzed using a fixed-effects model. Sensitivity analyses (on publication type, statistical model) were performed to further detect and evaluate clinically significant heterogeneity. Tests of survival for plasmablastic lymphoma were also performed by using Kaplan-Meier method. RESULTS Meta-analysis result showed that the prognosis of plasmablastic lymphoma patients was statistically different in the patients receiving HAART in addition to chemotherapy and/or radiotherapy than in the patients receiving the chemotherapy and/or radiotherapy alone (pooled relative risk=3.04; P=.03). Survival analyses also displayed a statistically significant difference (chi-square=6.22, P=.013). CONCLUSION HAART in addition to chemotherapy and/or radiotherapy is effective in improving the prognosis of plasmablastic lymphoma. However, the small sample sizes increase the likelihood of bias in the studies in this meta-analysis, and therefore, the results should be taken cautiously.
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Affiliation(s)
- Bing Guan
- Clinical School of Medical College of Nanjing University and Nanjing Jingling Hospital, Department of Pathology, Nanjing, China
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21
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Montes-Moreno S, Montalbán C, Piris MA. Large B-cell lymphomas with plasmablastic differentiation: a biological and therapeutic challenge. Leuk Lymphoma 2011; 53:185-94. [PMID: 21812534 DOI: 10.3109/10428194.2011.608447] [Citation(s) in RCA: 45] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
Plasmablastic differentiation can be found in a variety of large B-cell lymphomas, including plasmablastic lymphoma, ALK-positive large B-cell lymphoma, primary effusion lymphoma, large B-cell lymphoma arising in human herpesvirus-8 (HHV-8)-associated multicentric Castleman disease and diffuse large B-cell lymphoma (DLBCL) with partial plasmablastic phenotype. These tumors are characterized by acquisition of the transcriptional profile of plasma cells (with overexpression of PRDM1/Blimp1 and XBP1s, in concert with extinction of the B-cell differentiation program) by proliferating immunoblasts. This particular biological entity, i.e. large B-cell lymphoma with plasmablastic differentiation, is almost always associated with an aggressive clinical behavior. This review summarizes the current knowledge of the biological basis of plasmablastic differentiation in large B-cell lymphomas, the diagnostic borders with DLBCL and multiple myeloma, the associated adverse molecular events (with concomitant MYC, p53 and ALK alterations) and the potential therapeutic targets so far identified (including the unfolded protein response pathway). The highly aggressive nature of these lymphomas and the relative paucity of molecular data available highlight the need for deeper insights into the molecular pathogenesis of large B-cell lymphomas with plasmablastic differentiation in order to identify new and effective alternative treatments.
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22
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Boy SC, van Heerden MB, Babb C, van Heerden WF, Willem P. Dominant genetic aberrations and coexistent EBV infection in HIV-related oral plasmablastic lymphomas. Oral Oncol 2011; 47:883-7. [PMID: 21783402 DOI: 10.1016/j.oraloncology.2011.06.506] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2011] [Revised: 06/23/2011] [Accepted: 06/26/2011] [Indexed: 01/12/2023]
Abstract
We present common cytogenetic features in the largest cohort of plasmablastic lymphoma (PBL) of the oral cavity published to date. This cohort included 45 patients, 32 of whom had a known HIV status, of which 31 were HIV positive. Ninety eight per cent of all PBL cases were known to be EBV positive. In line with previous studies, we found that rearrangements of the MYC gene was the most common genetic abnormality seen in 60% of cases with the immunoglobulin heavy chain (IGH) locus as a partner in 51% of cases. Additional complex genetic aberrations were frequent, in particular, an increased copy number of the CCND1 gene was seen in 41% of cases with true amplification of CCND1 in 15% of cases. Aneuploidy was also observed for the BCL6 gene in 28% of cases. Interestingly, rearrangements of both IGH genes were detected in 16% of cases with t(14;18) and t(11;14) respectively involved in conjunction with a t(8;14) in two cases. These bi-allelic IGH rearrangements have not been described before in oral PBL. Our results reinforce the notion that EBV infection and MYC rearrangements are important events in the pathogenesis of oral PBL. The genetic diversity and complexity observed in these cases, underlines the importance to genetically characterise PBL patients at presentation as this may inform the choice of more effective treatment modalities.
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Affiliation(s)
- Sonja C Boy
- Department of Oral Pathology and Oral Biology, School of Dentistry, Faculty of Health Sciences, University of Pretoria, Pretoria, South Africa.
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23
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Bibas M, Grisetti S, Alba L, Picchi G, Del Nonno F, Antinori A. Patient With HIV-Associated Plasmablastic Lymphoma Responding to Bortezomib Alone and in Combination With Dexamethasone, Gemcitabine, Oxaliplatin, Cytarabine, and Pegfilgrastim Chemotherapy and Lenalidomide Alone. J Clin Oncol 2010; 28:e704-8. [DOI: 10.1200/jco.2010.30.0038] [Citation(s) in RCA: 70] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Affiliation(s)
- Michele Bibas
- National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy
| | - Susanna Grisetti
- National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy
| | - Lucia Alba
- National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy
| | - Giovanna Picchi
- National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy
| | - Franca Del Nonno
- National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy
| | - Andrea Antinori
- National Institute for Infectious Diseases Lazzaro Spallanzani, Rome, Italy
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24
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Hansra D, Montague N, Stefanovic A, Akunyili I, Harzand A, Natkunam Y, de la Ossa M, Byrne GE, Lossos IS. Oral and extraoral plasmablastic lymphoma: similarities and differences in clinicopathologic characteristics. Am J Clin Pathol 2010; 134:710-9. [PMID: 20959653 DOI: 10.1309/ajcpjh6keusecqlu] [Citation(s) in RCA: 51] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
Plasmablastic lymphoma (PBL), initially characterized as an aggressive lymphoma arising in the jaw and oral mucosa in HIV-infected patients, was recently reported to occur with extraoral manifestations, heterogeneous histologic findings, and variable association with immunodeficiency states. We reviewed clinical, morphologic, and immunophenotypic features of 13 cases of PBL to determine whether these different subtypes represent distinct morphologic and clinical entities. Two distinct subtypes of PBL were identified and classified as oral and extraoral PBL. The oral PBLs were strongly associated with HIV infection and commonly demonstrated plasmablastic morphologic features without plasmacytic differentiation. Extraoral PBLs tended to occur in patients with underlying non-HIV-related immunosuppression and universally demonstrated plasmacytic differentiation. The patients with oral PBL demonstrated better overall survival compared with patients with extraoral PBL (P = .02). Our findings suggest that PBL with oral and extraoral manifestation represent 2 distinct clinicopathologic entities.
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25
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Boy SC, Van Heerden MB, Raubenheimer EJ, Van Heerden WFP. Plasmablastic lymphomas with light chain restriction - plasmablastic extramedullary plasmacytomas? J Oral Pathol Med 2010; 39:435-9. [DOI: 10.1111/j.1600-0714.2009.00874.x] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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26
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Morley AMS, Verity DH, Meligonis G, Rose GE. Orbital Plasmablastic Lymphoma—Comparison of a Newly Reported Entity with Diffuse Large B-cell Lymphoma of the Orbit. Orbit 2009; 28:425-9. [PMID: 19929677 DOI: 10.3109/01676830903177427] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
MESH Headings
- Adult
- Antigens, CD/analysis
- Antineoplastic Combined Chemotherapy Protocols/therapeutic use
- Antiretroviral Therapy, Highly Active
- Biomarkers, Tumor/analysis
- Cyclophosphamide/therapeutic use
- Diagnosis, Differential
- Doxorubicin/therapeutic use
- Fatal Outcome
- HIV Infections/complications
- HIV Infections/drug therapy
- Humans
- Immunophenotyping
- Lymphoma, B-Cell/drug therapy
- Lymphoma, B-Cell/pathology
- Lymphoma, Large B-Cell, Diffuse/drug therapy
- Lymphoma, Large B-Cell, Diffuse/pathology
- Male
- Middle Aged
- Orbital Neoplasms/drug therapy
- Orbital Neoplasms/pathology
- Plasma Cells/pathology
- Prednisone/therapeutic use
- Tomography, X-Ray Computed
- Vincristine/therapeutic use
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