Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management

Author:

Ramirez‐Gamero Andres1ORCID,Martínez‐Cordero Humberto2ORCID,Beltrán Brady E.3ORCID,Florindez Jorge4ORCID,Malpica Luis5ORCID,Castillo Jorge J.16ORCID

Affiliation:

1. Division of Hematologic Malignancies Dana‐Farber Cancer Institute Boston Massachusetts USA

2. Instituto Nacional de Cancerologia and Hospital Militar Central Bogota Colombia

3. Department of Oncology and Radiotherapy, Hospital Edgardo Rebagliati Martins and Instituto de Ciencias Biomedicas Universidad Ricardo Palma Lima Peru

4. Division of Hematology and Oncology University of North Carolina Chapel Hill North Carolina USA

5. Department of Lymphoma and Myeloma University of Texas MD Anderson Cancer Center Houston Texas USA

6. Department of Medicine Harvard Medical School Boston Massachusetts USA

Abstract

AbstractDisease OverviewPlasmablastic lymphoma (PBL) is a rare CD20‐negative aggressive lymphoma with a poor prognosis under standard treatment options. Though PBL is associated with human immunodeficiency virus infection and other immunosuppressed states, it can also affect immunocompetent individuals.DiagnosisThe diagnosis requires a high clinical suspicion and pathological confirmation. EBER expression and MYC gene rearrangements are frequently detected. The differential diagnosis includes EBV+ diffuse large B‐cell lymphoma, extracavitary primary effusion lymphoma, ALK+ DLBCL, and HHV8+ large B‐cell lymphoma, among others.Risk StratificationAge ≥60 years, advanced clinical stage, and high intermediate and high International Prognostic Index scores are associated with worse survival.ManagementCombination chemotherapy regimens, such as EPOCH, are recommended. The addition of bortezomib, lenalidomide, or daratumumab might improve outcomes. Including PBL patients and their participation in prospective clinical trials is warranted.

Publisher

Wiley

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