POSTTRANSPLANTATION LYMPHOPROLIFERATIVE DISORDERS IN PEDIATRIC THORACIC ORGAN RECIPIENTS

Objective: To determine the frequency, predisposing factors, clinical presentation, and outcome of posttransplantation lymphoproliferative d isorders (PTLDs) in pediatric thoracic organ transplant recipients. Me thods: Retrospective review of the medical records of all 120 children who survived longer than 1 month after thoracic organ transplantation at our center. Results: PTLD was diagnosed in 14 patients (11.7%), in cluding 7.7% of heart and 19.5% of heart-lung/lung recipients. Present ation of PTLD was variable, ranging fr-om asymptomatic lung nodules on chest radiograph to diffuse multiorgan failure. Treatment with a redu ction of immunosuppression and antiviral therapy resulted in resolutio n of PTLD in eight patients. Eight patients died. PTLD contributed to death in five. No patient seropositive for Epstein-Barr virus (EBV) be fore transplantation had PTLD. There was a significant association bet ween primary EBV infection after transplantation and the presence of P TLD. Conclusions: PTLD occurs with greater frequency in pediatric thor acic organ transplant recipients than in the adult transplant populati on. Primary EBV infection after transplantation is the major risk fact or for the development of PTLD. Patients in whom primary EBV infection develops after transplantation should be managed with a reduction in immunosuppression and with heightened surveillance for the development of PTLD.