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Plasmablastic lymphoma

Mitsuhiro Akiyama, Tsutomu Takeuchi
IgG4-related disease is a heterogeneous immune-mediated fibroinflammatory condition that can affect every single organ. This disease is more prevalent in the elderly (the mean age of patients is above 60 years) and the prevalence rate is estimated to be over 4.6 per 100,000 population. Before making a diagnosis, the exclusion of malignancies, lymphoma, anti-neutrophil cytoplasmic antibody-associated vasculitis, multicentric Castleman disease, and other mimickers is crucial for appropriate treatment. Broad management guidelines have been published emphasizing the need for prompt treatment and the use of glucocorticoids as first-line drug therapy for induction of remission...
March 15, 2018: Drugs & Aging
Stefan D Dojcinov, Falko Fend, Leticia Quintanilla-Martinez
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...
March 7, 2018: Pathogens
Ming Hu, Jose Trevino, Lijun Yang, Dengfeng Cao, Xiuli Liu, Jinping Lai
Epstein-Barr virus (EBV)-positive diffuse large B cell lymphoma (DLBCL) of the elderly is a rare subtype of B-cell neoplasms. Primary gastric EBV-positive DLBCL of the elderly with partial plasmablastic phenotype is extremely rare. Differentiation of EBV-positive DLBCL of the elderly with partial plasmablastic phenotype from true plasmablastic lymphoma (PBL) is difficult and very important from the therapeutic and prognostic point of view. Here we report a case of a 59-year-old man with upper gastrointestinal bleeding...
March 2018: In Vivo
Maddalena Migliavacca, Andrea Assanelli, Maurilio Ponzoni, Roberta Pajno, Federica Barzaghi, Fabio Giglio, Francesca Ferrua, Marta Frittoli, Immacolata Brigida, Francesca Dionisio, Roberto Nicoletti, Miriam Casiraghi, Maria Grazia Roncarolo, Claudio Doglioni, Jacopo Peccatori, Fabio Ciceri, Maria Pia Cicalese, Alessandro Aiuti
Adenosine deaminase-deficient severe combined immunodeficiency disease (ADA-SCID) is a primary immune deficiency characterized by mutations in the ADA gene resulting in accumulation of toxic compounds affecting multiple districts. Hematopoietic stem cell transplantation (HSCT) from a matched donor and hematopoietic stem cell gene therapy are the preferred options for definitive treatment. Enzyme replacement therapy (ERT) is used to manage the disease in the short term, while a decreased efficacy is reported in the medium-long term...
2018: Frontiers in Immunology
Ayako Sakakibara, Kei Kohno, Ahmed E Eladl, Teerada Klaisuwan, Eri Ishikawa, Yuka Suzuki, Satoko Shimada, Masato Nakaguro, Yoshie Shimoyama, Taishi Takahara, Seiichi Kato, Naoko Asano, Shigeo Nakamura, Akira Satou
AIMS: The programmed death 1 (PD1)/PD1 ligand (PD-L1) axis plays an important role in tumour cells escape from immune control. PD-L1 immunohistochemistry is a useful predictor of immunotherapy response, but is still not widely used in the diagnostic setting. Here we describe results using PD-L1 immunohistochemistry during routine diagnostics in lymphoma. METHODS AND RESULTS: Ninety-one lymphoproliferative disease cases sharing tumour and nonmalignant Hodgkin/Reed-Sternberg (HRS)-like cells with and without Epstein-Barr virus (EBV) association were investigated by immunohistochemistry for PD-L1 (clone SP142)...
January 30, 2018: Histopathology
Edward Bloch, Fiona Robinson
Plasmablastic lymphoma is an unusual and aggressive form of diffuse large B-cell lymphoma, which arises more commonly within the oronasal mucosa. It should be considered as a differential diagnosis for rapidly growing periorbital lesions, particularly in the context of HIV positivity.
January 2018: Clinical Case Reports
Zenggang Pan, Mingyi Chen, Qianyun Zhang, Endi Wang, Liqun Yin, Youyuan Xu, Qin Huang, Youzhong Yuan, Xiaohui Zhang, Gang Zheng, Ji Yuan
Rare B-cell neoplasms with plasmablastic differentiation may aberrantly express CD3 by immunohistochemical staining, which places a great challenge for diagnosis. We here studied 17 cases of CD3+ plasmablastic B-cell neoplasms, including 12 plasmablastic lymphomas and 5 plasmablastic plasma cell myelomas. All 17 cases occurred in the extranodal sites with a male predominance (13/17). Four cases were initially misinterpreted by outside institutions, among which three were diagnosed as 'peripheral T-cell lymphoma, not otherwise specified' and one was classified as 'poorly differentiated neuroendocrine carcinoma'...
January 12, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Christopher Dittus, Natalie Grover, Steven Ellsworth, Xianming Tan, Steven I Park
Plasmablastic lymphoma (PBL) is a rare and aggressive form of B-cell non-Hodgkin lymphoma. This subtype of lymphoma has a post-germinal center cell-of-origin called the plasmablast, and the immunophenotype is more consistent with that of a plasma cell than a lymphocyte. Because of these unique features, PBL is notoriously difficult to treat. Case reports and small reviews have evaluated the addition of agents directed against plasma cell disorders in combination with traditional lymphoma-directed regimens. We describe the largest case series to date, with the longest follow-up, evaluating bortezomib in combination with etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (V-EPOCH) for the treatment of PBL...
January 5, 2018: Leukemia & Lymphoma
Maria Raffaella Ambrosio, Lucia Mundo, Sara Gazaneo, Matteo Picciolini, Prasad Satya Vara, Shaheen Sayed, Alessandro Ginori, Giuseppe Lo Bello, Leonardo Del Porro, Mohsen Navari, Stefano Ascani, Amhed Yonis, Lorenzo Leoncini, Pier Paolo Piccaluga, Stefano Lazzi
Plasmablastic lymphoma (PBL) is an aggressive lymphoma, often arising in the context of immunodeficiency and associated with Epstein-Barr virus (EBV) infection. The most frequently detected genetic alteration is the deregulation of MYC gene through the translocation - t(8;14)(q24;q32). The diagnosis of PBL is often challenging because it has an overlap in morphology, immunophenotype, cytogenetics and virus association with other lymphomas and plasma cell neoplasms; further, its molecular basis remains elusive...
December 8, 2017: Oncotarget
Bo-Jung Chen, Ran-Ching Wang, Chung-Han Ho, Chang-Tsu Yuan, Wan-Ting Huang, Sheau-Fang Yang, Pin-Pen Hsieh, Yun-Chih Yung, Shih-Yao Lin, Chen-Fang Hsu, Ying-Zhen Su, Chun-Chi Kuo, Shih-Sung Chuang
AIMS: To investigate the clinicopathological and molecular features of primary effusion lymphoma (PEL) in Taiwan and the association with human immunodeficiency virus (HIV), human herpesvirus 8 (HHV8) and Epstein-Barr virus (EBV). METHODS AND RESULTS: We investigated retrospectively 26 cases with a median age of 76.5. Only one (4%) patient was infected with HIV. Cytologically, all lymphoma cells revealed typical immunoblastic to plasmablastic morphology. Immunohistochemically, HHV8 was positive in eight (32%) tumours and negative in 17 (68%) cases...
December 5, 2017: Histopathology
Ita Hadžisejdić, Emina Babarović, Luka Vranić, Antica Duletić Načinović, Ksenija Lučin, Maja Krašević, Nives Jonjić
BACKGROUND: Plasmablastic lymphoma (PBL) is relatively new clinical entity described as a distinct subtype of diffuse large B-cell lymphoma (DLBCL). It is characterized by its aggressive nature and proliferation of large neoplastic cells resembling immunoblasts including cells with more obvious plasmacytic differentiation. In this case report, we describe an unexpected finding of PBL associated with a mature cystic teratoma of the ovary in a young immune competent woman. CASE PRESENTATION: A 19-year old woman was admitted to the hospital with generalized lymphadenopathy, a pelvic tumor mass measuring 35 × 30 cm and a 4 cm lump in her right breast...
November 29, 2017: Diagnostic Pathology
Wei Wang, Rashmi Kanagal-Shamanna, L Jeffrey Medeiros
AIMS: Lymphoproliferative disorders (LPD) characterised by human herpesvirus 8 (HHV8) and Epstein-Barr virus (EBV) infection are rare and two entities are included in the World Health Organisation classification: primary effusion lymphoma (PEL) and germinotropic LPD. These two entities have very different clinicopathological presentations and prognosis. Here we describe two cases of HHV8-positive, EBV-positive lymphoma with clinicopathological features that are not consistent with either PEL or germinotropic LPD...
November 3, 2017: Histopathology
Hind Rafei, Ehab El-Bahesh, Antoine Finianos, Min-Ling L Liu, Geraldine P Schechter
Clinical Practice Points. Plasmablastic lymphoma (PBL) is a rare and highly aggressive variant of diffuse large B cell lymphoma with median survival of advanced stage patients varying between 6 and 15 months in previous reports. We report here a human immunodeficiency virus-infected patient surviving over 12 years following treatment for advanced PBL with EPOCH chemotherapy and intrathecal therapy. This case highlights the potential for improved survival in PBL with intensive chemotherapy. Further, literature review suggests promising prospects utilizing novel targeted therapies to increase the rate of prolonged responses...
2017: Case Reports in Hematology
Brady E Beltran, Pilar Quiñones, Gadwyn Sanchez, Antonio Paredes, Celia Moises, Esther Cotrina, Carlos A Torres-Cabala, Roberto N Miranda, Jorge J Castillo
No abstract text is available yet for this article.
October 30, 2017: Leukemia & Lymphoma
Lindor Qunaj, Jorge J Castillo, Adam J Olszewski
Using records from the National Cancer Data Base, we studied overall survival of CD20-negative variants of diffuse large B-cell lymphoma (DLBCL): primary effusion (PEL, N = 228), plasmablastic (PBL, N = 481), ALK-positive large B-cell (ALK + LBLC, N = 15), and human herpesvirus-8-positive DLBCL (HHV8 + DLBCL, N = 77). Three-year survival was 27% for PEL, 40% for PBL, 34% for ALK + LBCL, and 63% for HHV8 + DLBCL. Compared with unspecified DLBCL, and adjusting for clinical characteristics (including the HIV status), survival was significantly worse for PEL (hazard ratio [HR], 1...
October 11, 2017: Leukemia & Lymphoma
Nivedita Arora, Arjun Gupta, Navid Sadeghi
Plasmablastic lymphoma (PBL) is an aggressive form of non-Hodgkin's lymphoma (NHL) classically seen in patients infected with the human immunodeficiency virus, but can also be seen in other immunocompromised states such as transplant recipients, autoimmune diseases and the elderly. PBL is generally associated with a poor prognosis despite chemotherapy. There is evidence supporting the use of bortezomib in combination with standard chemotherapy to achieve durable responses in patients with PBL. We describe a patient with acquired immunodeficiency syndrome who presented with rectal pain and bright red blood per rectum...
October 9, 2017: BMJ Case Reports
Badr AbdullGaffar, Rania M Seliem
CD20-negative diffuse large B-cell lymphomas (DLBCLs) constitute a rare and heterogeneous group of aggressive lymphomas. Known well-documented variants include plasmablastic lymphomas, primary effusion lymphomas, anaplastic kinase-positive large B-cell lymphomas, and large B-cell lymphomas arising in human herpesvirus 8 (HHV8)-associated multicentric Castleman disease. They impose diagnostic challenges for pathologists and therapeutic confrontations for clinicians. CD20 loss in B-cell lymphomas is a well-known phenomenon after rituximab therapy...
October 1, 2017: International Journal of Surgical Pathology
Thomas A Guerrero-Garcia, Renzo J Mogollon, Jorge J Castillo
Plasmablastic lymphoma (PBL) is a rare and hard to treat disease. With current standard chemotherapeutic regimens, PBL is associated with a median overall survival of 12-15 months. We performed a systematic review of the literature through March 31, 2017 looking for patients with a diagnosis of PBL who were treated with bortezomib, alone or in combination. We identified 21 patients, of which 11 received bortezomib in the frontline setting and 10 received bortezomib in the relapsed setting. Eleven patients were HIV-positive and 10 were HIV-negative...
November 2017: Leukemia Research
Carolline Fontes Alves Mariano, Glauce Lunardelli Trevisan, Antonio Augusto Velasco E Cruz, Fernando Chahud
Plasmablastic lymphoma (PBL) is a rare B-cell lymphoma occurring mainly in HIV patients. The tumor frequently involves extranodal sites such as the oral cavity, nasal cavity, gastrointestinal tract, skin, and lungs. The neoplastic cells are characterized by a plasmablastic appearance and typical immunophenotype that indicates plasma cell differentiation. Herein, we report a case of intraocular involvement by plasmablastic lymphoma in a HIV patient with a long history of cytomegalovirus retinitis and loss of vision...
2017: Case Reports in Pathology
Hossein Keyvani, Mohammad Hadi Karbalaie Niya, Maryam Esghaei, Farah Bokharaei-Salim, Seyed Hamid Reza Monavari
OBJECTIVE: Human herpesvirus 8 (HHV-8) is the causative agent of Kaposi's sarcoma (KS), but it has also been associated with different hematologic malignancies, including plasmablastic lymphoma, Multicentric Castleman's disease (MCD), primary effusion lymphoma (PEL) and various atypical lymphoproliferative disorders. Patients with underlying lymphoproliferative diseases and chronic blood disorders who become infected with this virus are at risk for human malignancies. This small study reported the frequency of human herpesvirus 8 in 81 Iranian patients with lymphoproliferative disorders for estimation of possible factors affecting malignancy...
October 2017: Microbial Pathogenesis
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