The Clinicopathologic Spectrum of Posttransplantation Lymphoproliferative Disorders

Abstract

Abstract Context.—Posttransplantation lymphoproliferative disorders (PTLDs) are a heterogeneous group of lymphoid proliferations occurring in the setting of solid organ or bone marrow transplantation. They show a clinical, morphologic, and molecular genetic spectrum ranging from reactive polyclonal lesions to frank lymphomas. The close association with Epstein-Barr virus has been established and the pathogenetic role of this virus is becoming better understood. Although they are relatively uncommon, PTLDs are a significant cause of morbidity and mortality in transplant patients. Objective.—To review the incidence, risk factors, clinical features, pathogenesis, and classification of PTLDs. Data Sources.—We reviewed relevant articles indexed in PubMed (National Library of Medicine), with emphasis on more recent studies. The classification of PTLDs is based on the most current World Health Organization classification text. Conclusions.—Posttransplantation lymphoproliferative disorders are a heterogeneous group of disorders showing a wide clinical and morphologic spectrum. Although relatively uncommon, PTLDs represent a serious complication after transplantation. Many risk factors for PTLD are well established, including transplanted organ, age at transplant, and Epstein-Barr virus seronegativity at transplant. However, other factors have been implicated and still require additional examination. Recent studies are shedding some light on the pathogenesis of PTLDs and defining relevant pathways related to Epstein-Barr virus. As the pathogenesis of PTLDs is further elucidated, the classification of PTLDs will most likely evolve.