Successful bone marrow transplantation with reduced intensity conditioning in a patient with delayed-onset adenosine deaminase deficiency.
Pediatr Transplant
; 17(1): E29-32, 2013 Feb.
Article
en En
| MEDLINE
| ID: mdl-22805442
ABSTRACT
In this case report, we describe successful BMT with RIC in a patient with delayed-onset ADA deficiency. A three-yr-old Japanese boy was diagnosed with delayed-onset ADA deficiency because of recurrent bronchitis, bronchiectasia, and lymphopenia. In addition, autoimmune thyroiditis and neutropenia were present. At four yr of age, he underwent BMT with a RIC regimen, including busulfan and fludarabine, from an HLA-identical healthy sister. Engraftment after BMT was uneventful without GVHD. Decreased ADA levels in blood immediately increased following BMT, and the patient was disease-free 13 months after BMT. These results suggest that BMT with RIC may sufficiently restore immune regulation in delayed-onset ADA deficiency. A longer follow-up period is needed to confirm these observations.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Adenosina Desaminasa
/
Trasplante de Médula Ósea
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Inmunodeficiencia Combinada Grave
/
Acondicionamiento Pretrasplante
/
Agammaglobulinemia
Límite:
Child, preschool
/
Humans
/
Male
Idioma:
En
Revista:
Pediatr Transplant
Asunto de la revista:
PEDIATRIA
/
TRANSPLANTE
Año:
2013
Tipo del documento:
Article
País de afiliación:
Japón