Bone marrow transplantation for Diamond-Blackfan anemia.
Bone Marrow Transplant
; 15(1): 55-8, 1995 Jan.
Article
em En
| MEDLINE
| ID: mdl-7742755
ABSTRACT
Diamond-Blackfan anemia (DBA) is a rare disorder usually diagnosed in the first year of life. Although most cases respond to corticosteroids, > 20% are, or become, steroid-resistant. We report 10 children with DBA who received a bone marrow transplant from an HLA-identical sibling (n = 8), maternal (n = 1) or unrelated (n = 1) donor and reported to the International Bone Marrow Transplant Registry. Among eight recipients of HLA-identical sibling transplants, six are alive 5-87 months after transplant with no evidence of DBA and with Karnofsky performance scores of 90-100%. The two recipients of non-HLA-identical sibling transplants died < 2 weeks after transplant. The actuarial 2-year probability of survival for the eight sibling transplants was 72 (37-92)% (95% confidence interval).
Buscar no Google
Base de dados:
MEDLINE
Assunto principal:
Transplante de Medula Óssea
/
Anemia de Fanconi
Idioma:
En
Ano de publicação:
1995
Tipo de documento:
Article
País de afiliação:
Estados Unidos