IgA Eksikliği ve Yaygın Değişken İmmün Yetmezlik

Yıl 2009, Cilt: 7 Sayı: 3, 90 - 95, 01.12.2009

Kadri Kamber Zuhal Karalı Sara Şebnem Kılıç

Öz

Selektif IgA eksikliği sIgAE ; sekretuvar IgA yokluğu ve serum IgA düzeyinin 5 mg/dl altında olması ile karakterize, en sık görülen immün yetmezliktir. IgAeksikliğinin patogenezi bilinmemekle birlikte, Ig sınıf değişimi ve sitokinlerindeiçinde olduğu izotip değişiminde anormallikler gösterilmiştir. Yaygın değişkenimmün yetmezlik YDİY , hipogamaglobulinemi, antikor yapımında bozukluk vetekrarlayan bakteriyel enfeksiyon ile karakterize, heterojen bir hastalık grubudur.Defektif T hücre aktivasyonu, tam fonksiyonel B hücre aktivasyonu için gerekliolan T ve B hücreler arasındaki ilişkiyi belirleyen CD40L ekspresyonundaki bozukluk ve/veya T hücre farklılaşmasında ihtiyaç duyulan sitokinlerin üretiminde anormalliklere yol açabilir. Bu da YDİY hastalarında bozulmuş sitokin üretimi açıklamalarından biri olan proliferasyon ve/veya farklılaşma bozukluklarına benzemektedir

Anahtar Kelimeler

Selektif IgA eksikliği, yaygın değişken immün yetmezlik, hipogamaglobulinemi

Selective IgA Deficiency and Common Variable Immunodeficiency

Öz

Selective IgA deficiency sIgAD , using 5 mg/dl of serum IgA as the upper limitfor diagnosis and concomitant lack of secretory IgA, is the most common formof 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 byhypogammaglobulinemia, impaired antibody production and recurrent bacterialinfections. Defective T-cell activation may lead to an impairment in cognate T-Bcell interaction due to impaired expression of CD40 ligand and/or abnormalitiesin the production of T-cell-derived cytokines required for fully functional B-cellactivation, proliferation and/or differentiation which could indeed explain theimpairment in antibody production present in CVID patients. It has been foundthat cytokines are produced in low levels due to the decreased T cell functionwhich occurs as a result of the defect in CD40L expression in CVID patients

Anahtar Kelimeler

Selective IgA deficiency, common variable immunodeficiency, hypogammaglobulinemia

Kaynakça

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