When immature plasma cells form lymphoma: how to improve on diagnostics and treatment of plasmablastic lymphoma?

Plasmablastic lymphoma (PBL) is a rare and aggressive, usually CD20-negative, B-cell lymphoma with features between multiple myeloma and diffuse large B-cell lymphoma. Approximately 120 new cases are diagnosed annually in the United States, and there is a strong association with HIV infection and other immunosuppressive states. It affects mostly adult males, though PBL has been diagnosed in children and immunocompetent individuals. Epstein-Barr virus infection, MYC gene rearrangements, and other mechanisms appear to play a role in PBL lymphomagenesis. Given its rarity, PBL poses distinct diagnostic and therapeutic challenges. A timely diagnosis is essential and requires a high clinical suspicion and a thorough pathological evaluation. The clinical course is aggressive, with a high relapse rate and poor survival with standard therapies. More recently, better outcomes have been observed in patients with early-stage disease treated with combination chemotherapy followed by consolidative radiotherapy. Additionally, advanced disease outcomes may improve with the use of targeted agents, such as proteasome inhibitors and anti-CD38 monoclonal antibodies, when added to combination chemotherapy. Participation in clinical trials and multi-institutional collaboration will be essential to continue improving patient outcomes with PBL.