HLA-haploidentical transplantations for primary immunodeficiencies: a single-center experience.
Pediatr Transplant
; 16(5): 451-7, 2012 Aug.
Article
em En
| MEDLINE
| ID: mdl-22594916
ABSTRACT
SCID is characterized by profound deficiencies of T and B lymphocytes. HSCT is the only curative treatment for children with SCID. The clinical characteristics and outcome of 30 HLA-haploidentical transplantations in 18 patients (15 SCID, two Omenn syndrome, and one MHC Class II deficiency) are reported here. The age of patients at diagnosis ranged from one and half to nine months (median four months). The median time was one month between the diagnosis and the time of the initial transplantation. Infused CD34+ stem cell dose was ranged between 7 and 94.2 × 10(6) /kg. Nine of 18 patients were found to be positive for CMV antigenemia at diagnosis; therefore, none of them received a conditioning regimen. The most common complication was graft failure (61%), so repeated transplantations (two to four) were performed in seven patients. The mean time of lymphoid engraftment was 17.5 days (median 16, range 11-29 days). Ten of 15 SCID (67%) patients survived with a stable complete donor chimerism. However, all three non-SCID patients died. In conclusion, in the absence of a matched family donor, HLA-haploidentical transplantation from parental donors represents a readily available treatment option especially for patients with SCID, offering a high chance of cure.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Imunodeficiência Combinada Severa
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Transplante de Células-Tronco Hematopoéticas
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Histocompatibilidade
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Antígenos HLA
Tipo de estudo:
Evaluation_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Female
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Humans
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Infant
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Male
Idioma:
En
Revista:
Pediatr Transplant
Assunto da revista:
PEDIATRIA
/
TRANSPLANTE
Ano de publicação:
2012
Tipo de documento:
Article
País de afiliação:
Turquia