Acquired Hemophilia A: An Update on the Etiopathogenesis, Diagnosis, and Treatment

Abstract

Acquired haemophilia A (AHA) is a rare bleeding disorder caused by inhibitory autoantibodies against coagulation factor VIII (FVIII). AHA is a disease that most commonly affects the elderly but has also been observed in children and in the postpartum period. AHA is idiopathic in 50% of cases and is associated with autoimmune diseases, malignancies, and infections in the remaining 50%. Recently, cases of association between AHA, COVID-19 vaccination, and infection have been reported in the literature. For diagnoses, determining FVIII levels is crucial to distinguish the different causes of aPTT prolongation. Treatment of AHA is based on bypassing agents (recombinant factor VIIa, activated prothrombin complex concentrate) and porcine FVIII to control the bleeding and immunosuppressive therapy (corticosteroids, rituximab, cyclophosphamide) to suppress autoantibody production. It is important to start a prophylactic regimen to prevent further bleeding episodes until the inhibitor is negative. Recently, the series of cases reported in the literature suggest that emicizumab may provide effective and safe haemorrhage prophylaxis in the outpatient setting.

Keywords: COVID-19; acquired hemophilia A; bypassing agents; emicizumab.

Figure 1

Flow chart for the diagnosis of AHA = acquired Hemophilia A. LA = lupus anticoagulant; PT = prothrombin time; TT = thrombin time; aPTT = activated partial prothrombin time; FVIII = Coagulation factor VII, IX =Factor IX, XI = Factor XI, XII = Factor XII.