Plasmablastic Lymphoma: 2026 Update on Diagnosis, Risk Stratification, and Management

Castillo J.J.; Beltrán B.E.; Florindez J.A.; Chavez J.C.; Malpica L. · American Journal of Hematology (2026) · DOI: 10.1002/ajh.70373

Resumen

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.

Instituciones: Universidad San Ignacio de Loyola