ABSTRACT
Epstein-Barr virus (EBV)–associated lymphoproliferative disorders were first identified as a complication of immunodeficiency in the setting of organ transplantation. Similar lymphoproliferations have since been recognized in a variety of acquired and congenital immunodeficiency states. The clinical, pathologic, and molecular features of these disorders differ significantly from those of non-Hodgkin’s lymphomas (HLs) encountered in immunocompetent individuals. Initially, these EBV-associated lymphoproliferations were viewed as EBV-associated B-cell tumors, but proliferations of T-cell origin and EBV-negative tumors have become increasingly common in such patients after survival for many years with immunodeficiency. The high prevalence in pediatric organ transplant recipients and following the use of intensive immunosuppressive regimens has also contributed to growing concerns about these disorders. Despite the curability of a proportion of patients, mortality from the disease has typically been high. Newer diagnostic and screening techniques and therapeutic advances such as monoclonal antibodies and adoptive T-cell therapy are likely to result in a lower prevalence and better treatment outcome.
