Lymphocytes and B-cell abnormalities in patients with common variable immunodeficiency (CVID)

Allergol Immunopathol (Madr). 2014 Jan-Feb;42(1):35-43. doi: 10.1016/j.aller.2012.07.016. Epub 2013 Jan 8.

Abstract

Background and aims: Common variable immunodeficiency (CVID) is a primary antibody deficiency characterised by decreased antibody production and low or normal B-cell numbers. To elucidate the clinical and immunological heterogeneity of CVID, we studied 16 patients diagnosed with CVID.

Methods: We analysed B, T and NK cell populations. We also assessed CD27 expression to define B-cell subsets and examined the expression of molecules important in B-cell proliferation and differentiation, such as the transmembrane activator and CALM interactor (TACI), inducible costimulator (ICOS), CD154 and CD40.

Results: We observed reduced B and T-cell numbers in CVID patients; this reduction was more pronounced in adults. While one group of patients (group I) showed a significant reduction in CD27+ memory B-cells, another group (group II) of patients exhibited numbers of CD27+ memory B-cells similar to the healthy donor. The frequency of B-cells and T-cells expressing CD40 and ICOS, respectively, was significantly lower in all CVID patients compared with healthy donors. Finally, a correlation between the frequency of CD27+ memory B-cells and clinical features was observed in CVID patients.

Conclusion: These results suggest that in some patients, the combined defects in both T and B-cells may account for CVID. Additionally, patients in group I exhibited an increased frequency of pneumonia and chronic diarrhoea.

Keywords: CD154; CD40; CVID; ICOS; Memory B-cells; TACI.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • B-Lymphocyte Subsets / immunology*
  • B-Lymphocytes / immunology*
  • CD40 Antigens
  • CD40 Ligand / metabolism
  • Child
  • Child, Preschool
  • Common Variable Immunodeficiency / immunology*
  • Female
  • Humans
  • Immunologic Memory
  • Inducible T-Cell Co-Stimulator Protein / genetics
  • Inducible T-Cell Co-Stimulator Protein / metabolism
  • Killer Cells, Natural / immunology*
  • Male
  • Mexico
  • Middle Aged
  • T-Lymphocytes / immunology*
  • Transmembrane Activator and CAML Interactor Protein / genetics
  • Transmembrane Activator and CAML Interactor Protein / metabolism
  • Tumor Necrosis Factor Receptor Superfamily, Member 7 / metabolism
  • Young Adult

Substances

  • CD40 Antigens
  • ICOS protein, human
  • Inducible T-Cell Co-Stimulator Protein
  • TNFRSF13B protein, human
  • Transmembrane Activator and CAML Interactor Protein
  • Tumor Necrosis Factor Receptor Superfamily, Member 7
  • CD40 Ligand