Post-Transplantation B Cell Function in Different Molecular Types of SCID
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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.
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.
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.
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
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- Post-Transplantation B Cell Function in Different Molecular Types of SCID
Journal of Clinical Immunology
Volume 33, Issue 1 , pp 96-110
- Cover Date
- Print ISSN
- Online ISSN
- Springer US
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- B cell function
- B cell chimerism
- bone marrow transplantation
- severe combined immunodeficiency
- molecular type
- memory B cells
- Industry Sectors
- Author Affiliations
- 1. Department of Pediatrics, Duke University Medical Center, Box 2898, 363 Jones Building, Durham, NC, 27710, USA
- 2. Department of Immunology, Duke University Medical Center, Durham, NC, 27710, USA
- 3. Department of Surgery, Duke University Medical Center, Durham, NC, 27710, USA
- 4. Department of Biostatistics and Bioinformatics, Duke University Medical Center, Durham, NC, 27710, USA