INTRODUCTION: Selective deficiency IgA (IgAD) is the most common primary abnormality of immunoglobulin production with unknown pathophysiology. It is genetically related to common variable immunodeficiency (CVID), where besides IgA also IgG and frequently IgM serum levels are decreased. In this study we focused on determination of B-lymphocyte developmental stages and searching for similarities between CVID and IgAD. MATERIALS AND METHODS: Using flow cytometry we determined major lymphocyte subpopulations and B-lymphocyte subsets: naïve (CD27(-)IgD(+)), marginal zone cells (CD27(+)IgD(+)), class-switched memory cells (CD27(+)IgD(-)), "double-negative" B cells (CD27(-)IgD(-)), transitional cells (IgM(++)CD38(++)), plasmablasts (CD38(+++)IgM(+) or IgM(-)), and CD21(low)CD38(low) cells in 80 patients with IgAD, 48 patients with CVID, and 80 control persons. RESULTS: Compared to healthy controls, a decrease in the absolute number and frequency of CD4+ cells (both < 0.001) was observed in IgAD patients. A decrease in the frequency of switched memory cells (P < 0.001), transitional cells (P = 0.035) as well as plasmablasts (P < 0.001) and an increase in the CD21(low)CD38(low) subset (P = 0.007) was observed in IgAD patients compared to control persons. No significant differences were observed in the remaining B-cell developmental subsets. A decrease in CD27(+)IgD(-) (<0.4% of peripheral blood lymphocytes), frequently observed in CVID patients and also previously reported in IgAD, was found in only five patients (6%) with IgAD, two of them being first-degree relatives of CVID patients. CONCLUSION: Our results show a decrease of terminally differentiated B-lymphocyte subsets in patients with IgAD, similar as previously found in patients with CVID, but these results are less expressed than in CVID patients.

Department of Clinical Immunology and Allergology St Anne's University Hospital Faculty of Medicine Masaryk University Pekarska 53 656 91 Brno Czech Republic

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J Immunol. 1995 Apr 1;154(7):3156-61 PubMed

Blood. 2008 Sep 1;112(5):1570-80 PubMed

Int Arch Allergy Immunol. 2000 Oct;123(2):177-80 PubMed

Clin Immunol Immunopathol. 1995 Nov;77(2):185-92 PubMed

Vaccine. 2011 May 31;29(24):4142-50 PubMed

Clin Immunol Immunopathol. 1996 Sep;80(3 Pt 1):333-5 PubMed

Clin Exp Immunol. 2006 Feb;143(2):373-9 PubMed

Clin Immunol. 2006 Sep;120(3):310-8 PubMed

Clin Immunol. 1999 Dec;93(3):190-7 PubMed

Clin Immunol. 1999 Jul;92(1):34-48 PubMed

Cytometry B Clin Cytom. 2008 Sep;74(5):261-71 PubMed

Clin Immunol. 2006 Nov;121(2):203-14 PubMed

Clin Exp Immunol. 2005 Jun;140(3):532-9 PubMed

Iran J Allergy Asthma Immunol. 2004 Sep;3(3):103-13 PubMed

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