Guidelines for the management of acquired hemophilia A in elderly patients

Abstract

Acquired hemophilia A is an autoimmune disease caused by antibodies against coagulation factor VIII (FVIII). These antibodies, called circulating FVIII anticoagulant or FVIII inhibitor, reduce plasma FVIII activity, which results in excessive bleeding. Acquired hemophilia A is classified as severe hemorrhagic disorder. Typical for this disease are extensive subcutaneous spontaneous blood extravasations, intramuscular and retroperitoneal hematomas, and post--traumatic bleeds, including post-surgery bleeding. In approximately 30% of cases, acquired hemophilia A is initially manifested merely by minor bleeding, which may be often overlooked by physician, but as long as FVIII inhibitor is detected, patient is at risk of severe, sometimes fatal, hemorrhage. Acquired hemophilia A is most common in people aged 60-90 years. Patients in this age group often take anticoagulants, antiplatelet and non-steroidal anti-inflammatory drugs, and bruising and purpura are relatively common among them, so acquired hemophilia A can easily be overlooked. On the other hand, the delay in the diagnosis of the disorder may result in delayed initiation of hemostatic treatment and elimination of the FVIII inhibitor, which puts the patient at risk of premature death. The aim of this publication is to present the principles of diagnosis of acquired hemophilia A, the use of hemostatic drugs to inhibit and prevent bleeding, and immunosuppressive drugs to eliminate the FVIII inhibitor in elderly patients.