Emicizumab in acquired haemophilia A: about two clinical cases and literature review

Abstract

Acquired haemophilia A (AHA) is a rare and severe haemorrhagic autoimmune disease caused by autoantibodies directed against factor VIII (FVIII). Treatment is based on two principles, including haemostatic control to compensate FVIII inhibition and eradication of inhibiting antibodies using immunosuppressive therapy. Rapid recognition and proper management are essential to avoid excess morbidity and mortality. Effective and safe treatments can be challenging, given that AHA patients are often elderly, with multiple comorbidities. Emicizumab, a bispecific antibody that mimics the action of FVIII, has proven effective in managing patients with congenital haemophilia, with or without inhibitors. Likewise, its mode of action suggests theoretical efficacy in AHA patients. We herein describe two AHA cases with comorbidities that were treated effectively using emicizumab combined with immunosuppressive therapy. We have also reviewed the current literature regarding the promising use of emicizumab in this indication.

Acquired Haemophilia A in four north European countries: survey of 181 patients

“…Furthermore, the bispecific antibody emicizumab is emerging as a potential treatment of AHA, with promising results. [31][32][33][34] As in previous studies, our data indicate a relationship between inhibitor titre and success rate of IST, 12 thus, stratification of patients into high-and low-risk according to baseline FVIII level and inhibitor titre for deciding on first-line IST, might also improve response rates and diminish side effects.…”

Acquired hemophilia as a rare cause of excessive bleeding during dentistry: report of two cases and short review

“…A future option for treatment of acquired hemophilia could also be the bispecific antibody emicizumab that allows to achieve adequate hemostasis in the absence of factor VIII. The agent has been approved for the treatment of inherited hemophilia A, and studies regarding its use in patients with acquired hemophilia have been initiated [8,9].…”

Current and future therapies for haemophilia—Beyond factor replacement therapies

“…Some reports demonstrate haemostatic effects following weekly emicizumab administration in patients with AHA 64–66 . Knoebl et al 65 assessed 12 patients with AHA treated with emicizumab (median age: 74 years).…”

“…Some reports demonstrate haemostatic effects following weekly emicizumab administration in patients with AHA. [64][65][66] Knoebl et al 65 assessed 12 patients with AHA treated with emicizumab (median age: 74 years). Emicizumab was initiated at 3 mg/kg weekly for two to three doses, followed by 1.5 mg/kg every three weeks.…”