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| Item Type: | Article |
|---|---|
| Title: | Altered B-cell subsets and functional B-cell defects in selective IgM deficiency |
| Creators Name: | Mensen, A., Krause, T., Hanitsch, L.G., Meisel, C., Kleint, M.E., Volk, H.D., 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 |
| 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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