Abstract Acquired haemophilia A (AHA) is an autoimmune bleeding disorder mediated by the production of autoantibody inhibitors against factor VIII (FVIII), resulting in a bleeding phenotype. The standard treatment of AHA includes immunosuppressive therapy for inhibitor eradication, haemostatic treatment with FVIII bypassing agents and treatment of underlying causes. Emicizumab is a bispecific monoclonal antibody that restores the function of missing activated FVIII by bridging FIXa and FX. Emicizumab is successfully used for prophylaxis of bleeding in congenital haemophilia A patients with or without FVIII inhibitors; however, it is not licensed for AHA treatment in the United Kingdom. We summarise our single-centre experience of the use of emicizumab for the treatment of AHA in 10 patients in the United Kingdom. Emicizumab was started at a dose of 3 mg/kg weekly with a change to fortnightly after the fourth dose. Eight patients received a combination of corticosteroids, emicizumab and rituximab; the remaining two, emicizumab and corticosteroids. In seven patients, emicizumab was stopped after achievement of an FVIII response and haemostasis following a median of 7 doses (3–53). In one patient, the treatment with emicizumab was complicated by the development of microangiopathic haemolytic anaemia. Three patients died, and in two of them, the death was associated with bleeding.
Tatarinova et al. (Tue,) studied this question.