Successful bone marrow transplantation in a Chinese boy with Wiskott-Aldrich syndrome.
Asian Pac J Allergy Immunol
;
1996 Jun; 14(1): 57-63
Artigo
em Inglês
| 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.
Texto completo:
DisponíveL
Índice:
IMSEAR (Sudeste Asiático)
Assunto principal:
Síndrome de Wiskott-Aldrich
/
Bussulfano
/
Humanos
/
Masculino
/
Transplante de Medula Óssea
/
Ciclofosfamida
/
Povo Asiático
/
Lactente
Idioma:
Inglês
Revista:
Asian Pac J Allergy Immunol
Ano de publicação:
1996
Tipo de documento:
Artigo
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