Posttransplant lymphoproliferative disorder.

OBJECTIVE: To define and discuss the pathogenesis, clinical presentation, diagnosis, risk factors, and current preventive and treatment strategies of posttransplant lymphoproliferative disorder (PTLD).

DATA SOURCES: MEDLINE was searched for articles published from January 1966 to July 2007. Search terms used include posttransplant lymphoproliferative disease, posttransplant malignancy, antiviral agents, interferon-alfa, rituximab, immunosuppression, chemotherapy, radiation, and surgery. Additional articles were identified by a hand search of references.

STUDY SELECTION AND DATA EXTRACTION: Studies in English of pediatric and adult solid organ transplantation populations published were selected and analyzed. Data from these studies and information from review articles were included in this review.

DATA SYNTHESIS: PTLD occurs in 1-20% of organ recipients following solid organ transplantation. PTLD risk factors include recipient pretransplant Epstein-Barr virus (EBV) negative serostatus, type of transplant, intensity of immunosuppression, and age. The PTLD presentation is variable. Some patients present asymptomatically; in others, early symptoms can be nonspecific. To prevent PTLD, minimizing immunosuppression burden and using antiviral agents active against EBV are useful strategies. PTLD treatment may require reduction of immunosuppression, radiation, surgical excision, monoclonal antibodies, interferon-alfa, and chemotherapy.

CONCLUSIONS: Screening for patients at risk and balancing the intensity of immunosuppressive regimens against the risk of rejection can substantially reduce the risk of developing PTLD. If PTLD occurs, an individualized treatment plan including decreased immunosuppression and other agents should be chosen based on the severity and extent of disease.