The management of posttransplant lymphoproliferative disorder

Posttransplant lymphoproliferative disorder (PTLD) is a life-threatening complication of allogeneic hematopoietic stem cell and solid organ transplantation. Most cases are EBV-positive B-cell neoplasms, which occur in the setting of pharmacologically impaired cellular immunity. Several different treatment strategies including cytotoxic antitumor therapy, anti-B-cell monoclonal antibody therapy, antiviral therapy, and modalities aimed at restoration of EBV-specific cellular immunity have been employed. In addition, efforts to identify patients at high risk for PTLD have resulted in attempts at prophylactic and preemptive therapies. In this review we discuss the available literature on differing approaches to PTLD management, identify areas in need of further investigation, and, when possible, make general recommendations. Reduction of immunosuppression remains the mainstay of first-line treatment. Accumulating evidence supports the role of rituximab as second-line therapy with cytotoxic chemotherapy reserved for specific circumstances. Further investigations are needed to better define the role of more novel and less widely available therapies such as the adoptive transfer of EBV-specific T cells and optimization of antiviral therapies.