Successful Treatment with Reduced Intensity Stem Cell Transplantation for a Boy with Chronic Active EB Virus Infection

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Chronic active EBV infection (CAEBV) is a poor prognostic disease, and allogeneic stem cell transplantation (allo-SCT) is one of the curative treatments. We report a boy with T cell-type CAEBV, who was successfully treated with reduced intensity stem cell transplantation (RIST). A 9-year-old boy was suffering from fever and hepatosplenomegaly, and was diagnosed with CAEBV based on a high level of EBV DNA in his plasma (1.5 ×106 copy/ml). Allogeneic bone marrow transplantation (BMT) from his HLA-matched mother was performed after a preconditioning regimen consisting of Fludarabine (30 mg/m2/day × 4 days), Melphalan (70 mg/m2/day × 2 days), and horse antithymocyte globulin (10 mg/kg/day × 2 days). He received an infusion of BM containing 3.3 × 108 mononuclear cells/kg. Cyclosporine A and short-term Methotrexate was used for prophylaxis against graft-versus-host disease (GVHD). He was engrafted with neutrophil > 5 × 102/μl on day 17, reticulocyte > 1.0% on day 21, and platelet > 5 × 104/μl on day 24, and complete chimera was confirmed by FISH analysis of sex chimerism on day 105. He developed grade II acute GVHD, and his hepatosplenomegaly subsided subsequently. EBV DNA became undetectable in the plasma after BMT. This outcome suggests that RIST could be a safe and effective treatment for the patients with CAEBV.
特定非営利活動法人　日本小児血液・がん学会の論文