Serial transplantation of mismatched donor hematopoietic cells between HLA-identical sibling pairs with congenital immunodeficiency: in vivo tolerance permits rapid immune reconstitution following T-replete transplantation without GVHD in the secondary recipient

Blood. 2006 Sep 15;108(6):2124-6. doi: 10.1182/blood-2006-03-009712. Epub 2006 May 25.

Abstract

We report serial transplantation procedures in 2 sets of brothers with X-linked primary immunodeficiency. The first boy in each family received a T-cell-depleted transplant from a mismatched donor. The recipients then acted as donors for T-replete transplantation of the "tolerized" graft into their HLA-identical brothers with the same disorder. Immune reconstitution was noted to occur at a significantly faster rate in the secondary recipients, and without the occurrence of graft-versus-host disease (GVHD), despite the presence of donor cells mismatched for 1 to 3 HLA antigens. This serial transplantation technique allows the primary recipient of HLA-mismatched donor cells to act as a functionally "HLA-matched" donor for subsequent affected siblings, and should be considered as a therapeutic option in families with congenital disorders.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Child, Preschool
  • Chromosomes, Human, X / genetics
  • Diseases in Twins / genetics
  • Diseases in Twins / immunology
  • Diseases in Twins / therapy
  • Female
  • HLA Antigens
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Pregnancy
  • Severe Combined Immunodeficiency / genetics
  • Severe Combined Immunodeficiency / immunology
  • Severe Combined Immunodeficiency / therapy*
  • Siblings
  • Transplantation, Homologous
  • Twins, Monozygotic
  • Wiskott-Aldrich Syndrome / genetics
  • Wiskott-Aldrich Syndrome / immunology
  • Wiskott-Aldrich Syndrome / therapy*

Substances

  • HLA Antigens