Selective IgM deficiency: clinical and laboratory features of 17 patients and a review of the literature

Kód výsledku v 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>

Nalezeny alternativní kódy

RIV/00216224:14110/17:00098289 RIV/00216208:11150/17:10365610 RIV/65269705:_____/17:00067306 RIV/00179906:_____/17:10365610 a 2 dalších

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

Selective IgM deficiency: clinical and laboratory features of 17 patients and a review of the literature

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

Selective IgM deficiency: clinical and laboratory features of 17 patients and a review of the literature

Popis výsledku anglicky

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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30102 - Immunology

Projekt

<a href="/cs/project/NV15-28541A" target="_blank" >NV15-28541A: Dysregulace imunitního systému: vlastnosti lymfocytů u pacientů s immunodeficiencí a autoimunitními projevy</a><br>

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2017

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Journal of Clinical Immunology

ISSN

0271-9142

e-ISSN

—

Svazek periodika

37

Číslo periodika v rámci svazku

6

Stát vydavatele periodika

US - Spojené státy americké

Počet stran výsledku

16

Strana od-do

559-574

Kód UT WoS článku

000407597600011

EID výsledku v databázi Scopus

2-s2.0-85025160272