Emicizumab in acquired haemophilia A: about two clinical cases and literature review. (August 2021)

• Record Type: Journal Article
• Authors: Hansenne, Amandine; Hermans, Cedric
• Is Part Of: Therapeutic advances in hematology. Volume 12(2021)
• Publication Date: 2021-08
• Subjects: acquired haemophilia A -- bypassing agents -- emicizumab; Hematology -- Periodicals; Hematologic Diseases -- therapy -- Periodicals; Hematology -- Periodicals; 616.15005
• DOI: 10.1177/20406207211038193
• Languages: English
• ISSNs: 2040-6207

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.