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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.

Original publication

DOI

10.1182/bloodadvances.2018026963

Type

Journal article

Journal

Blood Adv

Publication Date

26/02/2019

Volume

3

Pages

570 - 574

Keywords

Fetal Blood, Graft Rejection, Humans, Lymphocyte Count, Lymphopenia, Mucopolysaccharidosis I, Transplantation Conditioning