title: Post-transplantation B cell function in different molecular types of SCID.
abstract: OBJECTIVE
  Severe combined immunodeficiency (SCID) is a syndrome of diverse genetic cause characterized by profound deficiencies of T, B and sometimes NK cell function. Non-ablative HLA-identical or rigorously T cell-depleted haploidentical parental bone marrow transplantation (BMT) results in thymus-dependent genetically donor T cell development in the recipients, leading to a high rate of long-term survival. However, the development of B cell function has been more problematic. We report here results of analyses of B cell function in 125 SCID recipients prior to and long-term after non-ablative BMT, according to their molecular type.
  
  
  METHODS
  Studies included blood immunoglobulin measurements; antibody titers to standard vaccines, blood group antigens and bacteriophage Φ X 174; flow cytometry to examine for markers of immaturity, memory, switched memory B cells and BAFF receptor expression; B cell chimerism; B cell spectratyping; and B cell proliferation.
  
  
  RESULTS
  The results showed that B cell chimerism was not required for normal B cell function in IL7Rα-Def, ADA-Def and CD3-Def SCIDs. In X-linked-SCID, Jak3-Def SCID and those with V-D-J recombination defects, donor B cell chimerism was necessary for B cell function to develop.
  
  
  CONCLUSIONS
  The most important factor determining whether B cell function develops in SCID T cell chimeras is the underlying molecular defect. In some types, host B cells function normally. In those molecular types where host B cell function did not develop, donor B cell chimerism was necessary to achieve B cell function. 236 words.
authors.affiliation: Department of Pediatrics, Duke University Medical Center, Box 2898, 363 Jones Building, Durham, NC 27710, USA. buckl003@mc.duke.edu
authors.forename: Rebecca H
authors.initials: RH
authors.lastname: Buckley
authors.forename: Chan M
authors.initials: CM
authors.lastname: Win
authors.forename: Barry K
authors.initials: BK
authors.lastname: Moser
authors.forename: Roberta E
authors.initials: RE
authors.lastname: Parrott
authors.forename: Elisa
authors.initials: E
authors.lastname: Sajaroff
authors.forename: Marcella
authors.initials: M
authors.lastname: Sarzotti-Kelsoe
mesh_terms: D000328:Adult; D016175:B-Lymphocyte Subsets / Q000276:immunology* / Q000473:pathology / Q000637:transplantation*; D016026:Bone Marrow Transplantation / Q000276:immunology* / Q000379:methods / Q000473:pathology*; D002472:Cell Transformation, Viral / Q000276:immunology; D002478:Cells, Cultured; D002648:Child; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007223:Infant; D019169:Jurkat Cells; D008212:Lymphocyte Depletion; D017710:Lymphocyte Transfusion / Q000379:methods; D008297:Male; D011184:Postoperative Period; D011828:Radiation Chimera / Q000276:immunology; D016511:Severe Combined Immunodeficiency / Q000276:immunology* / Q000473:pathology* / Q000601:surgery; D032681:Spectral Karyotyping; D016176:T-Lymphocyte Subsets / Q000473:pathology; D018183:Transplantation Chimera / Q000276:immunology; D014407:Tumor Cells, Cultured