Chédiak-Higashi syndrome: hematopoietic chimerism corrects genetic defect.
Bone Marrow Transplant
; 27(11): 1211-3, 2001 Jun.
Article
em En
| MEDLINE
| ID: mdl-11551033
Chediak-Higashi syndrome is a rare autosomal recessive disorder, primarily affecting neutrophils, and is often lethal by the third decade of life. Bone marrow transplantation is the only curative therapy currently available. This case describes a child undergoing a bone marrow transplant from a matched sibling donor, resulting in hematopoietic chimerism with only a small percentage of donor neutrophils found long term. The presence of a small percentage of donor neutrophils has resulted in normal development and no increased incidence of infections. Hematopoietic chimerism offers a cure with a potential reduction in the side-effects that result from marrow transplantation and the associated preparative therapies.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome de Chediak-Higashi
/
Transplante de Medula Óssea
Limite:
Female
/
Humans
/
Infant
Idioma:
En
Ano de publicação:
2001
Tipo de documento:
Article