Zita Chovancova1,2, Pavlina Kralickova3, Alena Pejchalova4, Marketa Bloomfield5, Jana Nechvatalova6,7, Marcela Vlkova6,7, Jiri Litzman6,7. 1. Department of Clinical Immunology and Allergy, St. Anne's University Hospital in Brno, Pekarska 53, 65691, Brno, Czech Republic. zita.chovancova@fnusa.cz. 2. Faculty of Medicine, Masaryk University, Brno, Czech Republic. zita.chovancova@fnusa.cz. 3. Charles University in Prague School of Medicine and University Hospital, Institute of Clinical Immunology and Allergology, Hradec Kralove, Czech Republic. 4. Transfusion and Tissue Department, University Hospital Brno, Brno, Czech Republic. 5. Department of Immunology, Motol University Hospital, Prague, Czech Republic. 6. Department of Clinical Immunology and Allergy, St. Anne's University Hospital in Brno, Pekarska 53, 65691, Brno, Czech Republic. 7. Faculty of Medicine, Masaryk University, Brno, Czech Republic.
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 21low 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.
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 21low 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.
Authors: Christoph B Geier; Kai M T Sauerwein; Alexander Leiss-Piller; Isabella Zmek; Michael B Fischer; Martha M Eibl; Hermann M Wolf Journal: Front Immunol Date: 2018-12-18 Impact factor: 7.561
Authors: Jeroen D Langereis; Stefanie S Henriet; Saskia Kuipers; Corry M R Weemaes; Mirjam van der Burg; Marien I de Jonge; Michiel van der Flier Journal: J Clin Immunol Date: 2018-01-15 Impact factor: 8.317