Managing post-transplant lymphoproliferative disorder

Introduction: Post-transplant lymphoproliferative disorders (PTLD) constitute a heterogeneous group of lymphoproliferative diseases that occur in the setting of transplantation of either hematopoietic stem cells (HSCT) or solid organs (SOT), and results from the uncontrolled neoplastic proliferation of plasmacytic or lymphoid cells in the context of extrinsic immunosuppression after transplantation. PTLD in HSCT setting are largely caused by latent Epstein-Barr Virus (EBV).Areas covered: In patients after HSCT, EBV infections can cause life-threatening multiorgan disease. This review summarizes the incidence and risk factors of EBV-PTLD in transplant patients as well as the recently developed standards for diagnostic methods and strategies with respect to the predominant risk factors in adults and children. Recommendations for preemptive and targeted treatment strategies and outcomes in different risk groups are presented. Novel therapeutic approaches in the context of allogeneic HSCT, including rituximab and EBV-specific cytotoxic T lymphocytes (EBV-CTL), are discussed.Expert opinion: With the current diagnostic and therapeutic strategy, a positive outcome is reached in 70% of patients. Further research in the area should focus on resistant cases of PTLD, that is EBV-low expression PTLD, EBV-negative PTLD, T-PTLD, or composite B/T-PTLD. Research should also concentrate on molecular, proteomic, metabolic, or genomewide studies aiming to find new markers constituting possible new targets for personalized therapy.