title: Chimerism in children with primary immunodeficiencies is influenced by number of activating killer cell immunoglobulin-like receptor genes in the donor and/or killer cell immunoglobulin-like receptor ligand mismatch.
abstract: BACKGROUND
  Stem cell transplantation (SCT) is a curative treatment for children with primary immunodeficiencies.
  
  
  METHODS
  The present retrospective analysis describes the long-term outcomes at a median follow-up of 9 years of 29 patients with immunodeficiency after SCT; 5 sibling and 24 alternative donor transplantations. T-cell engraftment emphazed on thymic dependent signal-joint T-cell receptor excision circles (sjTREC) generation and donor chimerism in relation to killer cell immunoglobulin-like receptor genes and their ligands.
  
  
  RESULTS
  All children except two were reconstituted successfully from grafted material, including 9 and 18 cases of mixed chimerism (MC) and complete chimerism (CC), respectively. Univariate analyses showed that the number of activating KIR genes or HLA-C1/C2 ligand mismatches (P = .048) and possibly transplantation from an alternative donor (P = .054) facilitated CC development. Multivariate analysis showed that the presence of donor KIR haplotype B or incompatibility within C1/C2 ligands (relative risk, 6.1; 95% confidence interval, 1.08-34.69; P = .025) were significantly associated with the development of CC.
  
  
  CONCLUSIONS
  These results suggested that the donor-activating KIR gene repertoire affected successful engraftment.
authors.affiliation: L. Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, and Lower Silesian Center for Cellular Transplantation with National Polish Bone Marrow Donor Registry, Wroclaw, Poland. Electronic address: wysocz@iitd.pan.wroc.pl.
authors.forename: B
authors.initials: B
authors.lastname: Wysoczanska
authors.forename: K
authors.initials: K
authors.lastname: Koscinska
authors.forename: S
authors.initials: S
authors.lastname: Mizia
authors.forename: A
authors.initials: A
authors.lastname: Lange
mesh_terms: D002648:Child; D046528:Chimerism; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000276:immunology*; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D007694:Killer Cells, Natural / Q000276:immunology*; D008024:Ligands; D008297:Male
chemical_list: D007136:Immunoglobulins; D008024:Ligands