EBV-Associated Lymphoproliferative Disorders: Classification and Treatment

Abstract

Learning Objectives: After completing this course, the reader will be able to: Assess patients with EBV-associated lymphoproliferative disorders.Describe the pathogenesis of the lymphoproliferative disorders linked to EBV infection.Evaluate EBV cell–based immunotherapy for use in patients with EBV-associated lymphoproliferative disorders. CME This article is available for continuing medical education credit at CME.TheOncologist.com Since its discovery as the first human tumor virus, Epstein-Barr virus (EBV) has been implicated in the development of a wide range of B-cell lymphoproliferative disorders, including Burkitt's lymphoma, classic Hodgkin's lymphoma, and lymphomas arising in immunocompromised individuals (post-transplant and HIV-associated lymphoproliferative disorders). T-cell lymphoproliferative disorders that have been reported to be EBV associated include a subset of peripheral T-cell lymphomas, angioimmunoblastic T-cell lymphoma, extranodal nasal type natural killer/T-cell lymphoma, and other rare histotypes. EBV encodes a series of products interacting with or exhibiting homology to a wide variety of antiapoptotic molecules, cytokines, and signal transducers, hence promoting EBV infection, immortalization, and transformation. However, the exact mechanism by which EBV promotes oncogenesis is an area of active debate. The focus of this review is on the pathology, diagnosis, classification, and pathogenesis of EBV-associated lymphomas. Recent advances in EBV cell–based immunotherapy, which is beginning to show promise in the treatment of EBV-related disorders, are discussed.