Nonmyeloablative Bone Marrow Transplant from HLA-Identical Sibling for Chronic Granulomatous Disease

DOI 23 References
  • ONO Mayuko
    Department of Pediatrics, Hiroshima University School of Medicine
  • FUJITA Naoto
    Department of Pediatrics, Hiroshima Red Cross Hospital & Atomic-Bomb Survivors Hospital
  • HAMAMOTO Kazuko
    Department of Pediatrics, Hiroshima Red Cross Hospital & Atomic-Bomb Survivors Hospital
  • MIZUKAMI Tomoyuki
    Department of Pediatrics, Miyazaki Medical College, University of Miyazaki
  • NUNOI Hiroyuki
    Department of Pediatrics, Miyazaki Medical College, University of Miyazaki
  • KOBAYASHI Masao
    Department of Pediatrics, Hiroshima University School of Medicine

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  • 慢性肉芽腫症に対するＨＬＡ一致同胞からの骨髄非破壊的同種骨髄移植

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Chronic granulomatous disease (CGD) is an inherited disorder caused by the abnormalities of NADPH oxidase in phagocytic cells. Stem cell transplantation (SCT) from an HLA-identical donor is an effective treatment for patients with CGD. However, SCT with myeloablative conditioning carries a high incidence of serious complications and death. We report a 21-year-old man with CGD who was successfully treated with allogeneic bone marrow transplantation (alloBMT) from his HLA-identical brother following a nonmyeloablative conditioning regimen consisting of cyclophosphamide, fludarabine and a low dose of total body irradiation. Engraftment was rapidly achieved without any toxicity or complication. Partial donor chimerism following alloBMT was converted to complete donor chimerism and nomal oxidase activity of neutrophils was induced by several donor lymphocyte infusions (DLI) from his HLA-identical brother. At 30 months after transplant, the patient is in excellent clinical and hematological condition, maintaining complete chimerism. We suggest that alloBMT preceded by nonmyeloablative conditioning and followed by DLI may constitute a successful mode of therapy for patients with CGD.

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