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21.07.2017 | Original Article

Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature

verfasst von: Zita Chovancova, Pavlina Kralickova, Alena Pejchalova, Marketa Bloomfield, Jana Nechvatalova, Marcela Vlkova, Jiri Litzman

Erschienen in: Journal of Clinical Immunology | Ausgabe 6/2017

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Abstract

Purpose

Primary selective IgM deficiency (sIgMD) is a primary immunodeficiency with unclear pathogenesis and a low number of published cases.

Methods

We reviewed clinical and laboratory manifestations of 17 sIgMD patients. Serum IgM, IgG, and its subclasses, IgA, IgE, antibodies against tetanus toxoid, pneumococcal polysaccharides and Haemophilus influenzae type b, isohemagglutinins, and T and B lymphocyte subsets, expressions of IgM on B cells and B lymphocyte production of IgM were compared with previously reported case reports and a small series of patients, which included 81 subjects in total.

Results

We found that some patients in our cohort (OC) and published cases (PC) had increased IgE levels (OC 7/15; PC 21/37), decreased IgG4 levels (OC 5/14), very low titers of isohemagglutinins (OC 8/8; PC 18/21), increased transitional B cell counts (OC 8/9), decreased marginal zone B cell counts (OC 8/9), and increased 21^low B cell counts (OC 7/9). Compared with the PC (20/20), only two of five OC patients showed very low or undetectable production of IgM after stimulation. A majority of the patients had normal antibody production to protein and polysaccharide antigens, basic lymphocyte subset counts, and expression of surface IgM molecules on B cells.

Conclusions

Low IgM levels are associated with various immunopathological disorders; however, pathogenic mechanisms leading to decreased IgM serum level in selective IgM deficiency remain unclear. Moreover, it is difficult to elucidate how strong these associations are and if these immunopathological conditions are primary or secondary.

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Titel: Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature
verfasst von: Zita Chovancova
Pavlina Kralickova
Alena Pejchalova
Marketa Bloomfield
Jana Nechvatalova
Marcela Vlkova
Jiri Litzman
Publikationsdatum: 21.07.2017
Verlag: Springer US
Erschienen in: Journal of Clinical Immunology / Ausgabe 6/2017
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-017-0420-8

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Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature

Abstract

Purpose

Methods

Results

Conclusions

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