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

J 2017

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

CHOVANCOVÁ, Zita, Pavlina KRALICKOVA, Alena PEJCHALOVÁ, Marketa BLOOMFIELD, Jana NECHVÁTALOVÁ et. al.

Základní údaje

Originální název

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

Autoři

CHOVANCOVÁ, Zita (203 Česká republika, garant, domácí), Pavlina KRALICKOVA (203 Česká republika), Alena PEJCHALOVÁ (203 Česká republika), Marketa BLOOMFIELD (203 Česká republika), Jana NECHVÁTALOVÁ (203 Česká republika, domácí), Marcela VLKOVÁ (203 Česká republika, domácí) a Jiří LITZMAN (203 Česká republika, domácí)

Vydání

Journal of Clinical Immunology, New York, Springer, 2017, 0271-9142

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30102 Immunology

Stát vydavatele

Spojené státy

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 4.227

Kód RIV

RIV/00216224:14110/17:00098289

Organizační jednotka

Lékařská fakulta

DOI

http://dx.doi.org/10.1007/s10875-017-0420-8

UT WoS

000407597600011

Klíčová slova anglicky

Selective IgM deficiency; primary immunodeficiency; infections; autoimmunity; allergy

Štítky

EL OK

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 7. 3. 2018 16:08, Soňa Böhmová

Anotace

V originále

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

Citovat

CHOVANCOVÁ, Zita, Pavlina KRALICKOVA, Alena PEJCHALOVÁ, Marketa BLOOMFIELD, Jana NECHVÁTALOVÁ, Marcela VLKOVÁ a Jiří LITZMAN. Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature. Journal of Clinical Immunology. New York: Springer, 2017, roč. 37, č. 6, s. 559-574. ISSN 0271-9142. Dostupné z: https://dx.doi.org/10.1007/s10875-017-0420-8.

@article{1395614,
   author = {Chovancová, Zita and Kralickova, Pavlina and Pejchalová, Alena and Bloomfield, Marketa and Nechvátalová, Jana and Vlková, Marcela and Litzman, Jiří},
   article_location = {New York},
   article_number = {6},
   doi = {http://dx.doi.org/10.1007/s10875-017-0420-8},
   keywords = {Selective IgM deficiency; primary immunodeficiency; infections; autoimmunity; allergy},
   language = {eng},
   issn = {0271-9142},
   journal = {Journal of Clinical Immunology},
   title = {Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature},
   volume = {37},
   year = {2017}
}

TY  - JOUR
ID  - 1395614
AU  - Chovancová, Zita - Kralickova, Pavlina - Pejchalová, Alena - Bloomfield, Marketa - Nechvátalová, Jana - Vlková, Marcela - Litzman, Jiří
PY  - 2017
TI  - Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature
JF  - Journal of Clinical Immunology
VL  - 37
IS  - 6
SP  - 559-574
EP  - 559-574
PB  - Springer
SN  - 02719142
KW  - Selective IgM deficiency
KW  - primary immunodeficiency
KW  - infections
KW  - autoimmunity
KW  - allergy
N2  - Primary selective IgM deficiency (sIgMD) is a primary immunodeficiency with unclear pathogenesis and a low number of published cases. 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. 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. 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.
ER  -

CHOVANCOVÁ, Zita, Pavlina KRALICKOVA, Alena PEJCHALOVÁ, Marketa BLOOMFIELD, Jana NECHVÁTALOVÁ, Marcela VLKOVÁ a Jiří LITZMAN. Selective IgM Deficiency: Clinical and Laboratory Features of 17 Patients and a Review of the Literature. \textit{Journal of Clinical Immunology}. New York: Springer, 2017, roč.~37, č.~6, s.~559-574. ISSN~0271-9142. Dostupné z: https://dx.doi.org/10.1007/s10875-017-0420-8.

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