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

Shinjiro Todo Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan, 602

Fumihiro Fujiwara Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan, 602

Satoshi Ikushima Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan, 602

Takao Yoshihara Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan, 602

Yoshiko Kataoka Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan, 602

Yumiko Sakurai-Shimada Division of Pediatrics, Matsushita Memorial Hospital, Moriguchi, Osaka, Japan, 570

Koichi Sakata Division of Pediatrics, Matsushita Memorial Hospital, Moriguchi, Osaka, Japan, 570

Yasuro Kasubuchi Division of Pediatrics, Matsushita Memorial Hospital, Moriguchi, Osaka, Japan, 570

Yoshihito Morioka Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan, 602

Shinsaku Imashuku Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan, 602

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

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

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