# Emicizumab as first-line therapy in acquired hemophilia A

## TL;DR

Emicizumab, a new treatment for hemophilia, may be more effective and safer as a first-line therapy for acquired hemophilia A compared to current options.

## Contribution

The paper proposes emicizumab as a first-line treatment for acquired hemophilia A, potentially reducing bleeding and immunosuppressive therapy side effects.

## Key findings

- Emicizumab mimics FVIII and has shown effectiveness in managing bleeding in hemophilia patients.
- Using emicizumab early may reduce the need for intravenous bypassing agents and immunosuppressive therapy risks.
- Clinical experiences suggest emicizumab could be a safer alternative for acquired hemophilia A treatment.

## Abstract

Acquired hemophilia A (AHA) is a rare autoimmune disease resulting from the development of autoantibodies directed against endogenous factor (F)VIII, leading to bleeding manifestations that can be life-threatening. The current standard hemostatic treatment involves the use of bypassing agents that circumvent FVIII (recombinant activated FVII, activated prothrombin complex concentrate, and recombinant porcine FVIII) that must be administered intravenously and possess a short half-life. These limitations and the risk of potentially fatal bleeding complications justify the early initiation of immunosuppressive treatment (IST) aimed at promptly eradicating the autoantibodies. IST is not without side effects, sometimes severe and possibly fatal, especially in persons with AHA who are generally older and have multiple comorbidities. Emicizumab, a bispecific antibody that mimics the action of FVIII, has emerged as an effective hemostatic therapy among persons with congenital hemophilia, whether complicated by the presence of anti-FVIII antibodies or not. Numerous arguments from recent clinical experiences suggest positioning emicizumab as a first-line treatment for AHA. This strategy has the potential to reduce bleeding complications and, importantly, the side effects associated with IST, which can be delayed and tailored to each patient.