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Chédiak-Higashi syndrome: hematopoietic chimerism corrects genetic defect.
Trigg, M E; Schugar, R.
Afiliação
  • Trigg ME; Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
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.
Assuntos
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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
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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