CLASSIFICATION OF PRIMARY IMMUNODEFICIENCIES AS A REFLECTION OF MODERN IDEAS ABOUT THEIR PATHOGENESIS AND THERAPEUTIC APPROACHES

Abstract

Primary immune deficiencies (PID) are the heterogeneous group of genetically associated disorders developed due to defects of more than 300 different genes. Modern classification of PID based on main pathogenic mechanism of disease, but to a large extent relies on presence of any genetic defect. Thus, role of molecular and genetic methods of investigation of patients comes to the fore and often determines approaches to a target conservative or curative therapy of PIDs. Despite of successful application of new therapeutic approaches, therapy with the help of intravenous immune globulins remains actual for majority of patients with PID.