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

David Deambrosis, Su Han Lum, Ryan Malcolm Hum, Kay Poulton, Wendy Ogden, Simon Jones, Simon Stanworth, Denise Bonney, Prashant Hiwarkar, Robert F Wynn

Research output: Contribution to journalArticlepeer-review


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 languageEnglish
Pages (from-to)570-574
Number of pages5
JournalBlood Advances
Issue number4
Publication statusPublished - 26 Feb 2019


  • Fetal Blood/immunology
  • Graft Rejection/etiology
  • Humans
  • Lymphocyte Count
  • Lymphopenia/etiology
  • Mucopolysaccharidosis I/complications
  • Transplantation Conditioning/adverse effects


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