Plasmablastic lymphoma: current perspectives

Andres Lopez,1 Pau Abrisqueta2 1Lymphoma Unit, Department of Hematology, Vall d’Hebron University Hospital, Barcelona, Spain; 2Department of Hematology, Vall d’Hebron Institute of Oncology, Vall d’Hebron University Hospital, Barcelona, Spain Abstract: Plasmablastic lymp...

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Autores principales:	Lopez A, Abrisqueta P
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2018
Materias:	Plasmablastic lymphoma Review Diagnosis Treatment Diseases of the blood and blood-forming organs RC633-647.5
Acceso en línea:	https://doaj.org/article/617ed73d97d4453bb88fd0af0a7f21e9
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spelling	oai:doaj.org-article:617ed73d97d4453bb88fd0af0a7f21e92021-12-02T04:00:41ZPlasmablastic lymphoma: current perspectives1179-9889https://doaj.org/article/617ed73d97d4453bb88fd0af0a7f21e92018-10-01T00:00:00Zhttps://www.dovepress.com/plasmablastic-lymphoma-current-perspectives-peer-reviewed-article-BLCTThttps://doaj.org/toc/1179-9889Andres Lopez,1 Pau Abrisqueta2 1Lymphoma Unit, Department of Hematology, Vall d’Hebron University Hospital, Barcelona, Spain; 2Department of Hematology, Vall d’Hebron Institute of Oncology, Vall d’Hebron University Hospital, Barcelona, Spain Abstract: Plasmablastic lymphoma (PbL) is a rare and aggressive B-cell malignancy with large neoplastic cells, most of them resembling plasmablasts that have a CD20-negative phenotype. Although initially described as being associated with HIV, over the years it has also been identified in patients with solid organ transplant and immunocompetent patients. Little is known about molecular basis that drives PbL, and still its diagnosis remains challenging given its rarity. However, proper recognition of its clinical characteristics, localization, and morphological features can establish a correct diagnosis of PbL within the spectrum of CD20-negative large B-cell lymphomas (LBCLs). PbL is characterized by CD20 and PAX5 negativity together with the expression of CD38, CD138, MUM1/IRF4, Blimp1, and XBP1 plasmacytic differentiation markers. It is usually associated with Epstein–Barr virus infections, and MYC gene rearrangements. PbL should be carefully differentiated from other CD20-negative B-cell neoplasms, ie, primary effusion lymphoma, anaplastic lymphoma kinase-positive (ALK) large B-cell lymphoma, and LBCL in human herpesvirus 8-associated multicentric Castleman disease. Despite our improved understanding of this disease, its prognosis remains dismal with short overall survival. There is no standard of care for this entity. Several chemotherapy combinations have been used with hardly any differences on its outcome. Thus, new approaches with the addition of novel molecules are needed to overcome its poor prognosis. Our current understanding and knowledge of PbL relies primarily on case reports and small case series. In this review, we revise through an extensive literature search, the clinical and biological characteristics of this entity, and the potential therapeutic options. Keywords: plasmablastic lymphoma, reviewLopez AAbrisqueta PDove Medical PressarticlePlasmablastic lymphomaReviewDiagnosisTreatmentDiseases of the blood and blood-forming organsRC633-647.5ENBlood and Lymphatic Cancer: Targets and Therapy, Vol Volume 8, Pp 63-70 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Plasmablastic lymphoma Review Diagnosis Treatment Diseases of the blood and blood-forming organs RC633-647.5
spellingShingle	Plasmablastic lymphoma Review Diagnosis Treatment Diseases of the blood and blood-forming organs RC633-647.5 Lopez A Abrisqueta P Plasmablastic lymphoma: current perspectives
description	Andres Lopez,1 Pau Abrisqueta2 1Lymphoma Unit, Department of Hematology, Vall d’Hebron University Hospital, Barcelona, Spain; 2Department of Hematology, Vall d’Hebron Institute of Oncology, Vall d’Hebron University Hospital, Barcelona, Spain Abstract: Plasmablastic lymphoma (PbL) is a rare and aggressive B-cell malignancy with large neoplastic cells, most of them resembling plasmablasts that have a CD20-negative phenotype. Although initially described as being associated with HIV, over the years it has also been identified in patients with solid organ transplant and immunocompetent patients. Little is known about molecular basis that drives PbL, and still its diagnosis remains challenging given its rarity. However, proper recognition of its clinical characteristics, localization, and morphological features can establish a correct diagnosis of PbL within the spectrum of CD20-negative large B-cell lymphomas (LBCLs). PbL is characterized by CD20 and PAX5 negativity together with the expression of CD38, CD138, MUM1/IRF4, Blimp1, and XBP1 plasmacytic differentiation markers. It is usually associated with Epstein–Barr virus infections, and MYC gene rearrangements. PbL should be carefully differentiated from other CD20-negative B-cell neoplasms, ie, primary effusion lymphoma, anaplastic lymphoma kinase-positive (ALK) large B-cell lymphoma, and LBCL in human herpesvirus 8-associated multicentric Castleman disease. Despite our improved understanding of this disease, its prognosis remains dismal with short overall survival. There is no standard of care for this entity. Several chemotherapy combinations have been used with hardly any differences on its outcome. Thus, new approaches with the addition of novel molecules are needed to overcome its poor prognosis. Our current understanding and knowledge of PbL relies primarily on case reports and small case series. In this review, we revise through an extensive literature search, the clinical and biological characteristics of this entity, and the potential therapeutic options. Keywords: plasmablastic lymphoma, review
format	article
author	Lopez A Abrisqueta P
author_facet	Lopez A Abrisqueta P
author_sort	Lopez A
title	Plasmablastic lymphoma: current perspectives
title_short	Plasmablastic lymphoma: current perspectives
title_full	Plasmablastic lymphoma: current perspectives
title_fullStr	Plasmablastic lymphoma: current perspectives
title_full_unstemmed	Plasmablastic lymphoma: current perspectives
title_sort	plasmablastic lymphoma: current perspectives
publisher	Dove Medical Press
publishDate	2018
url	https://doaj.org/article/617ed73d97d4453bb88fd0af0a7f21e9
work_keys_str_mv	AT lopeza plasmablasticlymphomacurrentperspectives AT abrisquetap plasmablasticlymphomacurrentperspectives
_version_	1718401491152142336

Plasmablastic lymphoma: current perspectives

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