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Wolf HM, Thon V, Litzman J, et al. Detection of impaired IgG antibody formation facilitates the decision on early immunoglobulin replacement in hypogammaglobulinemic patients. Front Immunol. 2015;6:32doi: 10.3389/fimmu.2015.00032.
Wolf, H. M., Thon, V., Litzman, J., & Eibl, M. M. (2015). Detection of impaired IgG antibody formation facilitates the decision on early immunoglobulin replacement in hypogammaglobulinemic patients. Frontiers in immunology, 632. https://doi.org/10.3389/fimmu.2015.00032
Wolf, Hermann M, et al. "Detection of impaired IgG antibody formation facilitates the decision on early immunoglobulin replacement in hypogammaglobulinemic patients." Frontiers in immunology vol. 6 (2015): 32. doi: https://doi.org/10.3389/fimmu.2015.00032
Wolf HM, Thon V, Litzman J, Eibl MM. Detection of impaired IgG antibody formation facilitates the decision on early immunoglobulin replacement in hypogammaglobulinemic patients. Front Immunol. 2015 Feb 02;6:32. doi: 10.3389/fimmu.2015.00032. eCollection 2015. PMID: 25699049; PMCID: PMC4313720.
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Front Immunol. 2015 Feb 02;6:32. doi: 10.3389/fimmu.2015.00032. eCollection 2015.

Detection of impaired IgG antibody formation facilitates the decision on early immunoglobulin replacement in hypogammaglobulinemic patients.

Frontiers in immunology

Hermann M Wolf, Vojtech Thon, Jiri Litzman, Martha M Eibl

Affiliations

  1. Immunology Outpatient Clinic , Vienna , Austria.
  2. Department of Clinical Immunology and Allergology, Faculty of Medicine, Masaryk University , Brno , Czech Republic ; Department of Clinical Immunology and Allergology, St. Anne's University Hospital , Brno , Czech Republic.

PMID: 25699049 PMCID: PMC4313720 DOI: 10.3389/fimmu.2015.00032

Abstract

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.

Keywords: CVID; IVIG; IgG antibody deficiency; hypogammaglobulinemia; immunoglobulin treatment; primary vaccination

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