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Journal of Clinical Immunology
Published in: Journal of Clinical Immunology 1/2013

01-01-2013 | Original Research

Post-Transplantation B Cell Function in Different Molecular Types of SCID

Authors: Rebecca H. Buckley, Chan M. Win, Barry K. Moser, Roberta E. Parrott, Elisa Sajaroff, Marcella Sarzotti-Kelsoe

Published in: Journal of Clinical Immunology | Issue 1/2013

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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
Appendix
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Metadata
Title
Post-Transplantation B Cell Function in Different Molecular Types of SCID
Authors
Rebecca H. Buckley
Chan M. Win
Barry K. Moser
Roberta E. Parrott
Elisa Sajaroff
Marcella Sarzotti-Kelsoe
Publication date
01-01-2013
Publisher
Springer US
Published in
Journal of Clinical Immunology / Issue 1/2013
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI
https://doi.org/10.1007/s10875-012-9797-6

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