Unleashed platelets in aHUS

摘要

Several membrane and plasma proteins regulate the activity of the complement system. Deficiency of CD55 and CD59 on the cell surface causes paroxysmal nocturnal hemoglobinuria, which is characterized by intravascular hemolysis and recurrent venous and arterial thrombosis. Deficiency of factor H, a complement-regulatory plasma glycoprotein, is associated with a microan-giopathic hemolytic anemia indistinguishable from hemolytic uremic syndrome. This condition lacks a diarrheal prodrome and hence is known as atypical hemolytic uremic syndrome (aHUS). The structure of factor H comprises 20 homologous units known as short consensus repeats or sushi domains. The C-terminal domains of factor H contain the cell-surface binding sites; most of the mutations detected in aHUS patients are located within these domains. The role of factor H deficiency in pathogenesis of aHUS#