Immune cytopenia post-cord transplant in Hurler syndrome is a forme fruste of graft rejection.
Blood Adv
; 3(4): 570-574, 2019 02 26.
Article
en En
| MEDLINE
| ID: mdl-30787020
ABSTRACT
Umbilical cord blood (UCB) is the preferred donor cell source for children with Hurler syndrome undergoing transplant, and its use has been associated with improved "engrafted survival" rates. However, as in other pediatric recipients of UCB transplants for nonmalignant disease, immune-mediated cytopenia (IMC) is a significant complication. This article describes 8 episodes of IMC in 36 patients with Hurler syndrome undergoing UCB transplant. The incidence of IMC was increased in those with a higher preconditioning absolute lymphocyte count and in those conditioned with fludarabine-containing regimens rather than cyclophosphamide, and it included red cell alloantibodies directed at cord blood group antigens that are novel to the recipient. In several cases, IMC was a precursor to immune-mediated complete graft rejection. We describe IMC as part of a spectrum of graft rejection by a residual competent host immune system and a forme fruste of complete graft rejection.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Mucopolisacaridosis I
/
Acondicionamiento Pretrasplante
/
Sangre Fetal
/
Rechazo de Injerto
/
Linfopenia
Tipo de estudio:
Etiology_studies
Límite:
Humans
Idioma:
En
Revista:
Blood Adv
Año:
2019
Tipo del documento:
Article
País de afiliación:
Reino Unido