Post-transplantation lymphoproliferative disorders: advances in diagnosis, prevention and management in children.

With improving operative and postoperative survival after pediatric thoracic transplantation, attention has appropriately begun to focus on complications of long-term immunosuppression. One important complication is post-transplantation lymphoproliferative disorders. Much has been learnt about this spectrum of disorders over the last 15 years, including the pivotal role of primary Epstein-Barr virus infection in the etiology of most cases. Despite these advances, nomenclature remains confusing for the clinician, prediction of outcome is imprecise and treatment strategies are poorly defined. Indeed, no treatments have been subjected to comparative prospective clinical trials. Only recently has attention focussed on strategies for prevention. This article will review the current state of knowledge of post-transplantation lymphoproliferative disorders, with emphasis on recent advances in diagnosis, prevention and management.