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Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management
Andres Ramirez-Gamero1, Humberto Martínez-Cordero2, Brady E Beltrán3
1Division of Hematologic Malignancies, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
Disease Overview:
Plasmablastic 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.
Diagnosis:
The 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 Stratification:
Age ≥60 years, advanced clinical stage, and high intermediate and high International Prognostic Index scores are associated with worse survival.
Management:
Combination 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.
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