Selective IgM deficiency: clinical and laboratory features of 17 patients and a review of the literature
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11130%2F17%3A10365610" target="_blank" >RIV/00216208:11130/17:10365610 - isvavai.cz</a>
Alternative codes found
RIV/00216224:14110/17:00098289 RIV/00216208:11150/17:10365610 RIV/65269705:_____/17:00067306 RIV/00179906:_____/17:10365610 and 2 more
Result on the web
<a href="http://dx.doi.org/10.1007/s10875-017-0420-8" target="_blank" >http://dx.doi.org/10.1007/s10875-017-0420-8</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1007/s10875-017-0420-8" target="_blank" >10.1007/s10875-017-0420-8</a>
Alternative languages
Result language
angličtina
Original language name
Selective IgM deficiency: clinical and laboratory features of 17 patients and a review of the literature
Original language description
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.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30102 - Immunology
Result continuities
Project
<a href="/en/project/NV15-28541A" target="_blank" >NV15-28541A: Dysregulation of immune system: characteristics of lymphocytes in patients with immunodeficiency and autoimmunity</a><br>
Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2017
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Journal of Clinical Immunology
ISSN
0271-9142
e-ISSN
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Volume of the periodical
37
Issue of the periodical within the volume
6
Country of publishing house
US - UNITED STATES
Number of pages
16
Pages from-to
559-574
UT code for WoS article
000407597600011
EID of the result in the Scopus database
2-s2.0-85025160272