A practical approach to the patient with acquired haemophilia A in Poland

Streszczenie

Acquired haemophilia (AHA) is a severe acquired bleeding disorder that develops due to circulating autoantibodies directed against coagulation factor VIII (FVIII). These antibodies inhibit the coagulation activity of FVIII in patient’s plasma. AHA is mainly diagnosed in the elderly and more than half of the cases are idiopathic. Underlying conditions which favor AHA occurrence are — among others — autoimmune diseases, neoplasms, pregnancy and 12-month post-partum period. The clinical picture is dominated by extensive ecchymosis, less often by intramuscular and retroperitoneal haematomas or hemorrhages to the central nervous system. Laboratory findings include isolated prolonged activated partial thromboplastin time (APTT), decreased FVIII activity and the presence of a FVIII inhibitor. Although patients with AHA usually present with severe hemorrhagic disorders, asymptomatic forms may also occur, with accidentally detected prolonged APTT as the only abnormality. The aim of AHA management is bleeding control and eradication of inhibitor.