Disorders of Liver : Posttransplantation Lymphoproliferative Disorder After Liver Transplantation In Pediatric Patients

다국어 초록 (Multilingual Abstract)

Background/Aims: Posttransplantation lymphoproliferative disorder (PTLD) is a serious disease that has been defined as uncontrolled proliferation of lymphocytes in a context of posttransplant immunosuppression. Because it may progress to malignancy, PTLD is a life-threatening complication of organ transplantation in children. The aim of this study is to evaluate the clinical features, diagnosis, treatment and outcome of PTLD after liver transplantation in Korean children. Methods: From January 1988 to December 2006, 7 of 121 pediatric patients who underwent liver transplantation were diagnosed with PTLD. We retrospectively analyzed the age at transplantation, time of presentation after transplantation, clinical manifestations, histological findings, results of EBV assessments, managements and outcomes of PTLD. Results: The incidence of PTLD in liver transplant pediatric recipients was 5.8%. The mean (SD) age of patients at the time of liver transplantation was 8.57±4.65 months (range 4 to 18 months). PTLD were diagnosed less than 8 months after transplantation in five patients, at 41 months in one, and at 61 months in one. The common clinical presentations were persistent fever, diarrhea and hematochezia. Six of seven patients were found with EBV-positive. Histologic findings showed ``early lesion`` in two, polymorphic in two, and monomorphic in three patients. Burkitt lymphoma and lymphoblastic lymphoma were found in two of three monomorphic patients. All patients were treated with reduction of immunosuppression and infusion of gancyelovir. Rituximab was added in three patients. PTLD were successfully managed in all patients except one who died of sepsis during chemotherapy. Conclusions: PTLD is a fatal disease in liver transplant pediatric recipients. Early detection through the quantitative assay of EBV or gastrointestinal endoscopy, and adequate treatment such as reduction of immunosuppressant, antiviral agent and anti-CD 20 antibody therapy are important in the favorable outcome of PTLD.