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J Clin Immunol. 2013 Jan;33(1):96-110. doi: 10.1007/s10875-012-9797-6. Epub 2012 Sep 22.

Post-transplantation B cell function in different molecular types of SCID.

Author information

  • 1Department of Pediatrics, Duke University Medical Center, Box 2898, 363 Jones Building, Durham, NC 27710, USA. buckl003@mc.duke.edu

Abstract

PURPOSE:

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.

CONCLUSION:

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.

PMID:
23001410
[PubMed - indexed for MEDLINE]
PMCID:
PMC3549311
Free PMC Article

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