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Plasmablastic Lymphoma: 2026 Update on Diagnosis, Risk Stratification, and Management
Jorge J Castillo1, Brady E Beltrán2, Jorge A Florindez3
1Division of Hematologic Malignancies, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts, USA.
Disease Overview:
Plasmablastic lymphoma (PBL) is a rare CD20-negative aggressive lymphoma with poor prognosis when treated with standard treatment options. Although PBL is associated with HIV infection and other immunosuppressed states, it can also affect immunocompetent individuals.
Diagnosis:
The diagnosis of PBL requires a high clinical suspicion and pathological confirmation. EBV-encoded RNA (EBER) expression and MYC gene rearrangements are frequently detected in the malignant cells. The differential diagnosis includes EBV+ diffuse large B-cell lymphoma (DLBCL), extracavitary primary effusion lymphoma, ALK+ DLBCL, and HHV8+ large B-cell lymphoma, among others.
Risk Stratification:
Age ≥ 60 years, advanced clinical stage, and high International Prognostic Index scores are associated with worse survival.
Management:
Combination chemotherapy regimens, such as infusional EPOCH, are recommended. The addition of bortezomib or daratumumab might improve outcomes. B-cell maturation antigen-targeted therapies have shown early efficacy. The participation of patients with PBL in prospective clinical trials is warranted.