Immune cytopenia post-cord transplant in Hurler syndrome is a forme fruste of graft rejection

Blood Advances
David DeambrosisRobert F Wynn

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.

References

May 7, 2004·The New England Journal of Medicine·Susan L StabaJoanne Kurtzberg
Apr 14, 2009·Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation·Jaap Jan BoelensUNKNOWN EUROCORD, Inborn error Working Party of EBMT and Duke University
Mar 16, 2013·Blood·Jaap Jan BoelensUNKNOWN Centre for International Blood and Marrow Research
Dec 10, 2013·Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation·I H BartelinkJaap Jan Boelens
Jul 31, 2014·British Journal of Haematology·Jaap Jan BoelensRobert F Wynn
Feb 25, 2015·Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation·Mieke AldenhovenJaap Jan Boelens
Sep 3, 2017·Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation·Vanessa E KennedyMadan Jagasia

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