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@article{1317730,
author = {Wolf, Hermann M. and Thon, Vojtěch and Litzman, Jiří and Eibl, Martha M.},
article_location = {Lausanne},
article_number = {February 2015},
doi = {http://dx.doi.org/10.3389/fimmu.2015.00032},
keywords = {hypogammaglobulinemia; IgG antibody deficiency; CVID; immunoglobulin treatment; IVIG; primary vaccination},
language = {eng},
issn = {1664-3224},
journal = {Frontiers in Immunology},
title = {Detection of impaired IgG antibody formation facilitates the decision on early immunoglobulin replacement in hypogammaglobulinemic patients},
volume = {6},
year = {2015}
}
TY - JOUR
ID - 1317730
AU - Wolf, Hermann M. - Thon, Vojtěch - Litzman, Jiří - Eibl, Martha M.
PY - 2015
TI - Detection of impaired IgG antibody formation facilitates the decision on early immunoglobulin replacement in hypogammaglobulinemic patients
JF - Frontiers in Immunology
VL - 6
IS - February 2015
SP - 1-10
EP - 1-10
PB - Frontiers Research Foundation
SN - 16643224
KW - hypogammaglobulinemia
KW - IgG antibody deficiency
KW - CVID
KW - immunoglobulin treatment
KW - IVIG
KW - primary vaccination
N2 - Hypogammaglobulinemia (serum IgG lower than 2 SD below the age-matched mean) and clinical symptoms such as increased susceptibility to infection, autoimmune manifestations, granulomatous disease, and unexplained polyclonal lymphoproliferation are considered to be diagnostic hallmarks in patients with common variable immunodeficiency (CVID), the most frequent clinically severe primary immunodeficiency syndrome. In the present study, we investigated patients with hypogammaglobulinemia and no clinical or immunological signs of defective cell-mediated immunity and differentiated two groups on the basis of their IgG antibody formation capacity against a variety of different antigens (bacterial toxins, polysaccharide antigens, viral antigens). Patients with hypogammaglobulinemia and intact antibody production (HIAP) displayed no or only mild susceptibility to infections, while CVID patients showed marked susceptibility to bacterial infections that normalized following initiation of IVIG or subcutaneous immunoglobulin replacement therapy. There was a substantial overlap in IgG serum levels between the asymptomatic HIAP group and the CVID patients examined before immunoglobulin treatment. HIAP patients showed normal levels of switched B-memory cells (CD19(+)CD27(+)IgD(-)), while both decreased and normal levels of switched B-memory cells could be found in CVID patients. IgG antibody response to a primary antigen, tick-borne encephalitis virus (TBEV), was defective in CVID patients, thus confirming their substantial defect in IgG antibody production. Defective IgG antibody production against multiple antigens could also be demonstrated in an adult patient with recurrent infections but normal IgG levels. To facilitate early treatment before recurrent infections may lead to organ damage, the antibody formation capacity should be examined in hypogammaglobulinemic patients and the decision to treat should be based on the finding of impaired IgG antibody production.
ER -
WOLF, Hermann M., Vojtěch THON, Jiří LITZMAN and Martha M. EIBL. Detection of impaired IgG antibody formation facilitates the decision on early immunoglobulin replacement in hypogammaglobulinemic patients. \textit{Frontiers in Immunology}. Lausanne: Frontiers Research Foundation, 2015, vol.~6, February 2015, p.~1-10. ISSN~1664-3224. Available from: https://dx.doi.org/10.3389/fimmu.2015.00032.
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