Post-Transplant Lymphoproliferative Disorders

Post-transplant lymphoproliferative disorders (PTLDs) are a relatively rare but often devastating malignant complication of organ transplantation. PTLDs are a heterogenous group of polyclonal or monoclonal lymphoid cell proliferations seen in the context of post-organ transplant immunosuppression. Milder PTLD cases may be occasionally reversible by reduction or withdrawal of immunosuppression. More severe cases fall under Hodgkin’s or non-Hodgkin’s lymphoma types. Epstein-Barr (EB) virus infection is associated with the transformed cells in 50-70% of cases and > 90% of the B-cell lineage PTLD cases that occur early. Clinical presentation can be highly variable and non-specific, with tumor localization possible anywhere in the body. Histopathology is the gold standard for diagnosis, unless tissue is not accessible. Management strategies beyond reduction or discontinuation of immunosuppression involve anti-B-cell antibodies and lymphoma chemotherapy, typically nowadays in risk-adjusted sequential fashion. However, morbidity and mortality remain high. Preventive measures are limited, though preemptive interventions at the EB DNAemia stage can reduce the progression to EBV + PTLD.