Posttransplant lymphoproliferative disease following liver transplantation

Purpose of review: Despite contemporary immunosuppressive regimens, posttransplant lymphoproliferative disease (PTLD) remains a major complication after liver transplantation. This review highlights advances in the understanding of the pathophysiology, diagnosis, and management of PTLD in liver transplant recipients. Recent findings: The spectrum of PTLD after liver transplant ranges from polymorphic lymphoproliferation to high-grade monoclonal lymphoma and is usually related to outgrowth of lymphocytes infected with Epstein-Barr virus (EBV). Risk factors for PTLD include EBV-seronegativity of the recipient, young age, intensity of immunosuppression, and the first year posttransplant. Measurement of EBV load by quantitative polymerase chain reaction assays is an important aid in the surveillance and diagnosis of PTLD although the specificity for PTLD is only about 50% (specificity for EBV is ∼100%). In patients diagnosed with PTLD, management options include reduction of immunosuppression, rituximab, combination chemotherapy, and adoptive immunotherapy. Outcomes have improved because rituximab has been incorporated into treatment regimens, and immunotherapy approaches show promise. Summary: PTLD is a significant complication after liver transplantation, particularly in children. Advances in early detection approaches have aided in the diagnosis and management of PTLD, but further research to identify better predictive biomarkers is needed to improve risk-based treatment strategies.