Acquired Haemophilia A: An Intriguing Disease.

摘要: . Acquired Haemophilia A is a rare acquired bleeding disorder caused by Factor VIII autoantibodies, which neutralise FVIII activity. These inhibitors differ from alloantibodies against FVIII, can occur in congenital after repeated exposures to plasma-derived or recombinant products. In most cases, the disease occurs suddenly subjects without personal familiar history of bleedings, with symptoms that may be mild, moderate, severe. However, only laboratory alterations are present ~ 30% patients. The incidence varies 1 4 cases per million/year; more than 80% patients elderly, males and females similarly affected. There small peak related pregnancy young women aged 20-40 years. underdiagnosed elderly. diagnostic algorithm based on an isolated prolonged activated partial thromboplastin time, normal thrombin absence Lupus Anticoagulant, mixing test reveals presence inhibitor: finding reduced activity detection neutralising autoantibodies lead diagnosis. idiopathic 44%-63% while others etiological factors present. Bleeding prevention treatment therapeutic tools as by-passing agents, porcine concentrate or, limited number concentrates desmopressin. As soon diagnosis has been made, immunosuppressive therapy must started eradicate inhibitor. Better knowledge disease, optimal management eradication inhibitor have significantly morbidity mortality