Abstract
We report a 15-year-old boy who developed a rapidly progressive and fatal Epstein-Barr virus (EBV) associated lymphoproliferative disorder (LPD) after bone marrow transplantation. He underwent an unrelated bone marrow transplantation from a genotypically HLA 2-locus mismatched donor for acute lymphocytic leukemia in third complete remission. A conditioning regimen included antithymocyte globulin. He developed fungal septicemia on day 59 and acute graft-versus-host disease (GVHD) of the skin on day 68, followed by hepatosplenomegaly and edema in the face and the extremities. He presented with cervical swelling and dyspnea as a result of edema of the larynx on day 75 and was diagnosed with having monoclonal LPD. Prednisolone could not be discontinued because of the progression of acute GVHD. Acyclovir, large doses of intravenous immunoglobulin, and donor lymphocyte transfusion were not effective, and he died of septicemia on day 92. An autopsy specimen revealed extensive infiltration of lymphoma cells in multiple organs. Since monoclonal LPD can be rapidly progressive and fatal as in the present case, a monitoring of EBV genome in peripheral blood is indispensable for an early diagnosis and treatment in the LPD high-risk group.
