Serious bleeds in pediatric persons with hemophilia A on emicizumab prophylaxis

Res Pract Thromb Haemost. 2023 Oct 20;7(8):102238. doi: 10.1016/j.rpth.2023.102238. eCollection 2023 Nov.

Abstract

Background: Clinical trials have shown success in bleed prevention with emicizumab, but real-world data on the effectiveness of emicizumab in preventing serious bleeds in the pediatric population are lacking.

Objectives: To report real-world data on the effectiveness of Emicizumab in pediatric persons with hemophilia A.

Methods: We completed a retrospective chart review of 37 pediatric male patients aged ≤18 years on emicizumab prophylaxis for a median duration of 30.5 months at Children's Medical Center in Dallas, Texas.

Results: We identified 4 pediatric persons with severe hemophilia A with and without inhibitors who experienced a provoked or unprovoked serious bleed requiring hospitalization.

Conclusion: This study highlights that serious bleeds, both provoked and unprovoked, can occur in pediatric persons with severe hemophilia A. These findings are important for clinicians to provide appropriate counseling/education and recommendation of treatment for pediatric persons with severe hemophilia A through shared decision making. Up-titration of emicizumab or factor VIII replacement needs consideration in persons with hemophilia with suboptimal bleeding control or who participate in activities categorized as moderate- to high-risk activities.

Keywords: bleed; emicizumab; hemophilia A; pediatric; prophylaxis.