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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, roč. 37, č. 6, s. 559-574. ISSN 0271-9142. doi:10.1007/s10875-017-0420-8. 2017.

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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  -

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
Originální 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
Změnil	Změnila: Soňa Böhmová, učo 232884. Změněno: 7. 3. 2018 16:08.

Anotace
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.

Anotace

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.