Posttransplant Lymphoproliferative Disease in Pediatric Liver Transplant Recipients

Posttransplant lymphoproliferative disease was first reported in 1968. Posttransplant lymphoproliferative disease encompasses a range of abnormalities from benign infectious mononucleosis-like illnesses to non-Hodgkin's lymphomas with nodal and extranodal site involvement. We evaluated five children who had posttransplant lymphoproliferative disease after liver transplantation. Since 2001, we have performed 118 liver transplantations in 115 children. Five children (4.6%), including three girls and two boys of overall mean age, 3.9 year, developed posttransplant lymphoproliferative diseases. The indications for liver transplant were hepatoblastoma in one recipient and cholestatic liver disease in the remaining four subjects. Posttransplant lymphoproliferative disease was diagnosed at 6, 11, 17, 22, and 27 months after the liver transplantation. Imaging modalities identified generalized lymphadenopathy in one, multiple liver masses in one, a large portal mass in one, multiple stomach ulcers in one, and a large mediastinal mass in one recipient. At diagnosis, the recipient with the large mediastinal mass displayed cough; the remaining four recipients were asymptomatic. Histological findings showed B-cell lymphomas in three recipients and T-cell lymphomas in two. The results of in situ hybridization for Epstein-Barr virus were negative in one recipient and positive in four. Four recipients were treated with chemotherapy; the remaining recipient was treated with anti-CD20 monoclonal antibodies. The one recipient who had a large mediastinal mass died at 2 months after receiving the diagnosis of chemotherapy-related sepsis; the remaining four children are alive at 9, 11, 18, and 34 months after treatment. Our rate of posttransplant lymphoproliferative disease was similar to that in the literature. From a few months to several years after liver transplantation, radiologists must be alert to the possibility of posttransplant lymphoproliferative disease. Thorough imaging is required to detect the wide variety of potential presentations.