Selective IgA Deficiency and Common Variable Immunodeficiency


Kamber K., Karal Z., KILIÇ S. Ş.

GUNCEL PEDIATRI-JOURNAL OF CURRENT PEDIATRICS, vol.7, no.2, pp.90-95, 2009 (Journal Indexed in ESCI) identifier identifier

  • Publication Type: Article / Review
  • Volume: 7 Issue: 2
  • Publication Date: 2009
  • Title of Journal : GUNCEL PEDIATRI-JOURNAL OF CURRENT PEDIATRICS
  • Page Numbers: pp.90-95
  • Keywords: Selective IgA deficiency, common variable immunodeficiency, hypogammaglobulinemia, PRIMARY ANTIBODY DEFICIENCIES, SUBCUTANEOUS IMMUNOGLOBULIN, CLINICAL MANIFESTATIONS, MUTATIONS, TACI, HYPOGAMMAGLOBULINEMIA, INTERLEUKIN-4, SURVEILLANCE, REPLACEMENT, ACTIVATION

Abstract

Selective IgA deficiency (sIgAD), using 5 mg/dl of serum IgA as the upper limit for diagnosis and concomitant lack of secretory IgA, is the most common form of primary immunodeficiency. The pathogenesis of IgA deficiency is not known, although abnormalities in Ig class switching and the cytokines involved in isotype switching have been implicated. Common Variable Immunodeficiency (CVID) is a heterogenous group of B cell deficiency syndromes characterized by hypogammaglobulinemia, impaired antibody production and recurrent bacterial infections. Defective T-cell activation may lead to an impairment in cognate T-Bcell interaction due to impaired expression of CD40 ligand and/or abnormalities in the production of T-cell-derived cytokines required for fully functional B-cell activation, proliferation and/or differentiation which could indeed explain the impairment in antibody production present in CVID patients. It has been found that cytokines are produced in low levels due to the decreased T cell function which occurs as a result of the defect in CD40L expression in CVID patients.