Post-transplant lymphoproliferative disorders after solid organ transplantation in children

Post-transplant lymphoproliferative disease (PTLD) is a well recognized complication of solid organ transplantation (SOT) or bone marrow transplantation (BMT) associated with therapeutic immunosuppression (IS), first reported in 1968. Risk factors, therapy, and outcomes differ between PTLD observed following BMT and SOT. PTLD is a potentially fatal complication in the clinical course of transplant recipients, representing the most common malignancy after SOT in children and the second in the adult setting. This review presents the predisposing risk factors to the development of PTLD, along with clinical aspects, diagnostic work-up and therapeutic options in order to obtain a durable and complete remission with minimal toxicity. The extreme diversity of clinical presentations, sometimes with rapidly aggressive evolution, together with the heterogeneity of imagistic and histological findings, have proven the importance of the high degree of clinical suspicion. The early recognition and the prompt adequate treatment may improve the outcome.