Emicizumab bij verworven hemofilie A: Een overbrugging tot immunologische remissie?

Acquired haemophilia A (AHA) is a rare autoimmune bleeding disorder characterized by circulating inhibiting antibodies against endogenous coagulation factor VIII (FVIII), resulting in a decreased activity of factor VIII. As a consequence, patients have an increased risk of spontaneous bleeding. The management of AHA is challenging and consists of controlling and preventing bleeding in combination with eradication of inhibitory antibodies with immunosuppressive therapy. Current guidelines recommend treatment of minor and major bleedings with high dose recombinant FVIII or porcine FVIII, desmopressin and/or bypassing agents. Although effective in bleeding control, long-term prophylaxis is difficult to achieve. In this case report we report on the role of emicizumab (Hemlibra®) in controlling bleeding in a patient with AHA awaiting immunological response. Emicizumab is a bispecific antibody mimicking factor VIII function. As it is not affected by circulating auto-antibodies, it can be considered in patients with acquired haemophilia A to prevent bleeding awaiting
immunological response.