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KMID : 0387820030100020282
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Clinical Pediatric Hematology-Oncology
2003 Volume.10 No. 2 p.282 ~ p.286
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A Case of Allogeneic Stem Cell Transplantation for Chronic Granulomatous Disease
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Lee Jae-Hee
Lee Mee-Jung
Choi Eun-Seok
Hong Soo-Jong
Park Chan-Jeoung
Seo Jong-Jin
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Abstract
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Chronic granulomatous disease (CGD) is a rare primary immunodeficiency in children caused by an abnormal function of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase in the phagocytic cells, which results in an increased susceptibility to severe bacterial and fungal infections. Prophylactic trimethoprim-sulfamethoxazole improves medium-term survival, but cannot prevent inflammatory sequelae. It still shows high morbidity and mortality. Bone marrow transplantation (BMT) is currently the only curative treatment for CGD. We report on a 29- month-old boy with CGD who was successfully treated with allogeneic BMT from an HLA- identical unrelated donors following a conditioning regimen consisting of busulfan and cyclophosphamide. One year after post-transplantation, the boy is in excellent clinical and hematological condition with complete chimerism and normal nitroblue tetrazolium (NBT) test.
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KEYWORD
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Bone Marrow Transplatation, Chronic Granulomatous Disease, Children
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