[Hereditary complement defects]

Abstract

The complement system is one of the most important and best analyzed biological amplification systems in humans. Hereditary deficiency states of one of the components may lead to various autoimmune disorders, recurrent bacterial infections or recurrent cutaneous edema in non-atopic subjects. This review makes an attempt to describe clinics and pathophysiology of the component deficiencies known to date. A procedure for laboratory evaluation of patients with suspected complement deficiency is described.