J Clin Immunol (2017) 37:559–574 DOI 10.1007/s10875-017-0420-8 ORIGINAL ARTICLE Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature 1,2 3 4 5 Zita Chovancova & Pavlina Kralickova & Alena Pejchalova & Marketa Bloomfield & 1,2 1,2 1,2 Jana Nechvatalova & Marcela Vlkova & Jiri Litzman Received: 8 March 2017 /Accepted: 6 July 2017 /Published online: 21 July 2017 Springer Science+Business Media, LLC 2017 Abstract showed very low or undetectable production of IgM after Purpose Primary selective IgM deficiency (sIgMD) is a pri- stimulation. A majority of the patients had normal antibody mary immunodeficiency with unclear pathogenesis and a low production to protein and polysaccharide antigens, basic lym- number of published cases. phocyte subset counts, and expression of surface IgM mole- Methods We reviewed clinical and laboratory manifestations cules on B cells. of 17 sIgMD patients. Serum IgM, IgG, and its subclasses, Conclusions Low IgM levels are associated with various im- IgA, IgE, antibodies against tetanus toxoid, pneumococcal munopathological disorders; however, pathogenic mecha- polysaccharides and Haemophilus influenzae type b, nisms leading to decreased IgM serum level in selective IgM isohemagglutinins, and T and B lymphocyte subsets, expres- deficiency remain unclear. Moreover, it is
Journal of Clinical Immunology – Springer Journals
Published: Jul 21, 2017
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