[Acquired haemophilia: Update in 2024]

Abstract

Acquired hemophilia A (AHA) is a rare autoimmune disorder due to autoantibodies against factor VIII, with a high mortality risk. It should be suspected in subjects with abnormal bleedings, especially subcutaneous bleed associated with prolonged activated partial thromboplastin time (aPTT). AHA is often idiopathic but is associated with autoimmune diseases, malignancies, pregnancy and postpartum period or drugs. Treatment is based on haemostatic agents as by-passants agents such as factor VIIa and activated prothrombine concentrate complex or recombinant porcine factor VIII for severe bleeding. Eradication of inhibitor should be established as soon as the diagnosis is confirmed with steroid alone often associated with cytotoxic agents or rituximab, depending on FVIII activity and inhibitor titer. The purpose of this review is to summarize the epidemiology, etiopathogenesis, diagnosis, treatment of AHA and discuss current recommendations.

Keywords: Acquired hemophilia A; Activated prothrombin concentrate complex; Concentré de complexe prothrombique activé; Emicizumab; Eptacog alfa activated; Eptacog alpha activé; Hémophilie acquise; Immunosuppressive treatement; Rituximab; Susoctocog alpha; Traitement immunosuppresseur.