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Successful bone marrow transplantation in a Chinese boy with Wiskott-Aldrich syndrome.
Asian Pac J Allergy Immunol ; 1996 Jun; 14(1): 57-63
Artículo en Inglés | IMSEAR | ID: sea-36734
ABSTRACT
We describe the successful use of HLA-compatible sibling bone marrow transplantation (BMT) in a 17-month-old Chinese boy in whom Wiskott-Aldrich syndrome (WAS) was diagnosed on the basis of eczema, thrombocytopenia, recurrent otitis media and abnormal immunological tests. The conditioning chemotherapy included 2 days' oral busulfan, 40 mg/m2/6 hours, and 2 days' intravenous cyclophosphamide, 60 mg/kg/day (BU2CY2). Complete hematological chimerism was achieved 3 weeks after transplantation. Eight months after his BMT the eczema has resolved, platelet count is normal, and he no longer has frequent infections. BU2CY2 as a preconditioning regimen gave complete lymphohematopoietic engraftment in this WAS patient with no evidence of graft-versus-host disease. The excellent clinical response of this patient and the inevitable fatal outcome of WAS support the opinion that where a histocompatible donor is available, BMT at the earliest opportunity is the best option. We believe this is the first case of successful BMT in a Chinese patient with WAS.
Asunto(s)
Texto completo: Disponible Índice: IMSEAR (Asia Sudoriental) Asunto principal: Síndrome de Wiskott-Aldrich / Busulfano / Humanos / Masculino / Trasplante de Médula Ósea / Ciclofosfamida / Pueblo Asiatico / Lactante Idioma: Inglés Revista: Asian Pac J Allergy Immunol Año: 1996 Tipo del documento: Artículo

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Texto completo: Disponible Índice: IMSEAR (Asia Sudoriental) Asunto principal: Síndrome de Wiskott-Aldrich / Busulfano / Humanos / Masculino / Trasplante de Médula Ósea / Ciclofosfamida / Pueblo Asiatico / Lactante Idioma: Inglés Revista: Asian Pac J Allergy Immunol Año: 1996 Tipo del documento: Artículo