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Altered B-cell subsets and functional B-cell defects in selective IgM deficiency

Item Type:Article
Title:Altered B-cell subsets and functional B-cell defects in selective IgM deficiency
Creators Name:Mensen, A. and Krause, T. and Hanitsch, L.G. and Meisel, C. and Kleint, M.E. and Volk, H.D. and Na, I.K. and Scheibenbogen, C.
Abstract:Primary selective IgM deficiency (sIgM) is characterized by diminished serum IgM, infections and autoimmunity. Although there is some evidence of B-cell defects the pathogenesis of sIgM is poorly understood. We determined peripheral B-cell subsets and IgM-expression levels in 31 adult sIgM patients by flow cytometry. In a subset of patients B-cell subset alterations and antibody-secreting cells were determined by flow cytometry and ELISpot assay after in vitro differentiation. Patients had significantly increased transitional, decreased IgM only, switched and non-switched memory B cells and decreased membrane IgM-expression levels on memory B-cell subsets compared to healthy controls. A strongly diminished B-cell differentiation and expansion capacity was observed in 5/6 investigated patients. Severely reduced IgM-secreting capacity was detected in 2/6 patients. Taken together, our results show altered B-cell subsets and severe functional B-cell defects in sIgM. This may provide a diagnostic tool and basis for subclassification of patients to study the pathogenetic background.
Keywords:Selective IgM Deficiency, Primary Immunodeficiency, Transitional B cells, Memory B Cells, ELISpot Assay, Antibody-Secreting Cells
Source:Clinical Immunology
ISSN:1521-6616
Publisher:Elsevier / Academic Press (U.S.A.)
Volume:161
Number:2
Page Range:96-102
Date:December 2015
Official Publication:https://doi.org/10.1016/j.clim.2015.08.017
PubMed:View item in PubMed

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