A Case of Familial Erythrophagocytic Lymphohistiocytosis in Remission for Longer than 3 Years due to Chemotherapy and Allogeneic Bone Marrow Transplantation

Abstract

A boy with familial erythrophagocytic lymphohistiocytosis (FEL) is reported. His two siblings had died of the same disease. He was diagnosed at the age of 1 month and was treated with an exchange transfusion and chemotherapy consisting of VP16, prednisolone and intrathecal methotrexate. He attained complete remission. Four months later in remission, he received bone marrow transplantation (BMT) from an HLA-identical brother. The conditioning for BMT included intrathecal methotrexate 12 mg/m²/day×1, busulfan 4 mg/kg/day×4, VP16 60 mg/kg/day×1, cyclophosphamide 60 mg/kg/day×2 and anti-human lymphocyte globulin (AHLG) 30 mg/kg/day×3. cyclosporin A and MTX were used for the prophylaxis of GVHD. After BMT leukocytes counts increased to more than 1, 000/μ1 by day+18, and his blood type became identical with that of the donor at day+50. Grade 2 GVHD was treatable with prednisolone. His NK cell activity was very low at the onset of the disease, but improved after the transplant. Currently over 3 years old, he remains free of disease.