Via practica 3/2006

PRIMARY IMMUNE DEFICIENCIES, DIAGNOSIS AND THERAPY

Immunodeficiency is a quantitative or qualitative failure of immune system functions that results in a clinical presentation of increased susceptibility to infections, increased autoimmune manifestation or lymphoproliferative disease. Today we identify more than hundred primary immune deficiencies and 40 % of these inherited disorders are diagnosed after the 15th year of life. Negative family history does not exclude such an error. Most prevalent primary immune deficiencies are defects of antibody production, mostly selective IgA deficiency and common variable immunodeficiency (CVID). Review focus on description of basic characteristics, diagnosis and therapeutic approach.

Keywords: primary immune deficiencies, common variable immunodeficiency, diagnosis, therapy.