Allogeneic Bone Marrow Transplantation for Familial Erythrophagocytic Lymphohistiocytosis, with High Dose VP16-Containing Conditioning Regimen

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Abstract
A 6-month-old boy with familial erythrophagocytic lymphohistiocytosis (FEL) received allogeneic bone marrow transplantation (BMT) from an HLA-identical brother, after first achieving remission with Etoposide (VP16) and prednisone. The conditioning regimen for BMT consisted of high dose busulfan (16 mg/kg), cyclophosphamide (120 mg/kg), high dose VP16 (60 mg/kg) and intrathecal methotrexate and was well tolerated. Engraftment was achieved, and grade II GVHD was controlled with prednisone. Impaired NK activity present on admission gradually improved after BMT. The patient continues to be in complete remission more than 10 months after BMT.

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