Plasmablastic lymphoma (PBL) is recognized by the World Health Organization as a very aggressive subtype of non-Hodgkin lymphoma. It was initially described in the setting of human immunodeficiency virus (HIV) infection, but it has since been identified in immunocompetent patients, as well. PBL is characterized by CD20 negativity and is associated with Epstein-Barr virus infection. The outcome with available therapy is poor, with median survival of less than 1 year. Multiple adverse prognostic factors have been identified, including HIV-negativity, MYC gene rearrangement, high-risk international prognostic index, and not achieving complete remission after induction therapy. The role of intensification of induction chemotherapy is controversial. Novel agents have shown some activity in relapsed setting and may have a role in upfront line of treatment. The outcome for relapsed PBL is dismal. Autologous hematopoietic cell transplantation (AHCT) appears to be feasible and may produce better results than chemotherapy, but definitive data are sparse. Chemosensitivity before transplantation might be required to benefit from such therapy. Some data suggest a better outcome of PBL if consolidation with AHCT is used in first-line setting, particularly for those with high-risk disease.
Keywords: Autologous hematopoietic cell transplantation; CD20-negative lymphoma; Epstein-Barr virus; HIV-related lymphoma; MYC rearrangement; Non-Hodgkin lymphoma; Plasmablastic lymphoma.
Copyright © 2014 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.