Selective IgA deficiency and common variable immunodeficiency/ IgA eksikligi ve yaygin degisken immun yetmezlik

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Date: Sept. 2009
Publisher: Galenos Yayinevi Tic. Ltd.
Document Type: Clinical report
Length: 3,676 words

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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-13cell 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. Key words: Selective IgA deficiency, common variable immunodeficiency, hypogammaglobulinemia Selektif IgA eksikligi (sIgAE); sekretuvar IgA yoklugu ve serum IgA duzeyinin 5 mg/dl altinda olmasi ile karakterize, en sik gorulen immun yetmezliktir. IgA eksikliginin patogenezi bilinmemekle birlikte, Ig sinif degisimi ve sitokinlerinde icinde oldugu izotip degisiminde anormallikler gosterilmistir. Yaygin degisken immun yetmezlik (YDIY), hipogamaglobulinemi, antikor yapiminda bozukluk ve tekrarlayan bakteriyel enfeksiyon ile karakterize, heterojen bir hastalik grubudur. Defektif T hucre aktivasyonu, tam fonksiyonel B hucre aktivasyonu icin gerekli olan T ve B hucreler arasindaki iliskiyi belirleyen CD40L ekspresyonundaki bozukluk ve/veya T hucre farklilasmasinda ihtiyac duyulan sitokinlerin uretiminde anormalliklere yol acabilir. Bu da YDIY hastalarinda bozulmus sitokin uretimi aciklamalarindan biri olan proliferasyon ve/veya farklilasma bozukluklarina benzemektedir. Anahtar kelimeler: Selektif IgA eksikligi, yaygin degisken immun yetmezlik, hipogamaglobulinemi

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Gale Document Number: GALE|A209697432