Autoimmune Coagulopathies
摘要
Autoimmune coagulopathies (AiC)Autoimmune coagulopathies (AiC) are rare, acquired bleeding disorders that are characterized by the development of autoantibodies directed to a coagulation factor. These antibodies cause immune-mediated depletion or inhibition of coagulation factors, and consequently, increase the risk of bleeding. AiC have been described in almost all coagulation factors but the most frequently targeted is factor VIII (acquired hemophilia A), followed by von Willebrand factor (vWF)von Willebrand factor (vWF) (acquired von Willebrand disease). Clinically, they are characterized by the sudden onset of bleeding episodes in individuals without previous history of bleeding disorder and their treatment consists of control of bleeding with hemostatic therapy, eradication of the inhibitor mainly with immunosuppressive therapy and treatment of the underlying disease if any.