Vaskulárna medicína 1/2021

Acquired hemophilia A

Acquired hemophilia A is a rare autoimmune disease caused by the production of autoantibodies to own coagulation factor (F) VIII. In the laboratory, the diagnosis of acquired hemophilia is confirmed by determining low levels of FVIII and the presence of neutralizing antibodies, so-called FVIII inhibitor. In contrast to genetically caused hemophilia, it may manifest as relatively dramatic, often lifethreatening bleeding. It is often overlooked and underdiagnosed in routine clinical practice. The authors present a case of a 67-year-old patient with a chronic, insufficiently therapeutically controlled systemic connective tissue disease who developed acquired hemophilia A. A multidisciplinary approach is important in the presence of AHA in patients with systemic connective tissue disease.

Keywords: acquired hemophilia A, FVIII inhibitor, immunosuppression, connective tissue diseases