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KMID : 0191120080230010146
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Journal of Korean Medical Science
2008 Volume.23 No. 1 p.146 ~ p.148
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Unrelated Bone Marrow Transplantation with a Reduced Toxicity Myeloablative Conditioning Regimen in Wiskott-Aldrich Syndrome
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Kang Hyoung-Jin
Shin Hee-Young
Ahn Hyo-Seop
Kim Joong-Gon
Rhim Jung-Woo
Kim Eun-Kyung
Ko Sun-Hie
Park Jeong-Ah
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Abstract
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Wiskott-Aldrich syndrome (WAS) is an X-linked congenital immune-deficiency syndrome, and bone marrow transplantation (BMT) has become a curative modality. However, the transplant with the alternative donor needed more intensive conditioning with increased treatment-related toxicities. Recently, fludarabine-based reduced toxicity myeloablative conditioning regimens have been developed for adult myeloid malignancies with promising results of good engraftment and low treatment-related toxicities. To increase the engraftment potential without serious complications, a boy with WAS received successful unrelated BMT with a reduced toxicity myeloablative conditioning regimen composed of fludarabine (40 mg/m(2) on days -8, -7, -6, -5, -4, -3), busulfan (0.8 mg/kg i. v. q 6 hr on days -6, -5, -4, -3), and thymoglobulin (2.5 mg/kg on days -4, -3, -2). This novel conditioning regimen could improve the outcome of allogeneic transplantation for other non-malignant diseases such as congenital immune-deficiency syndromes or metabolic storage diseases.
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KEYWORD
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Wiskott-Aldrich Syndrome, Bone Marrow Transplantation, Busulfan, Fludarabine
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