LIMITS AND POSSIBILITIES IN A PRIMARY SELECTIVE IgA DEFICIENCY TREATMENT – CASE PRESENTATION

Abstract

The primary deficit of IgA is the most common congenital immunodeficiency defined by the values of serum IgA below 0.07 g/l and normal values of IgG and IgM. Most cases are asymptomatic, only 10-15% is manifested by recurrent or persistent sinopulmonary infections, autoimmune diseases, allergies, malignancies. Some cases develop over the time a common variable immunodeficiency. The frequency of anti-IgA antibody justifies the cautiously use of transfusions of blood, plasma or intravenous immunoglobulin. The authors present a case of primary selective IgA deficiency, where the severity of infectious exacerbations appears to be due to the combination of a lack of IgG4, and discuss the possibilities and limits of therapy in light of recent data from the literature.

Keywords

• primary selective iga deficiency
• igg4 subclass