Supplementary Material for: A Jehovah's Witness with acquired hemophilia A who successfully avoided blood transfusions by receiving emicizumab

Introduction: Acquired hemophilia A (AHA) manifests as a significant deficiency in coagulation factor VIII (FVIII) activity caused by the development of autoantibodies against FVIII and results in severe bleeding symptoms that can be life-threatening. Therefore, patients with AHA often require blood transfusions. Emicizumab, a bispecific monoclonal antibody that replaces the function of activated FVIII, has been more widely used to treat AHA.
Case Presentation: We report a 70-year-old male Jehovah's Witness with AHA who refused blood transfusions despite experiencing severe bleeding in the left biceps muscle; anemia had progressed by approximately 100x104/μL in just 2 days. Since the use of coagulation factor products had been approved in writing, we administered a single-dose mixture of activated factor VIIa/X (Byclot®) and promptly achieved hemostasis. Subsequently, regular administration of emicizumab was effective in preventing rebleeding and successfully avoiding blood transfusions.
Conclusion: For Jehovah's Witnesses with AHA, the administration of emicizumab can be considered a highly valuable treatment.

引言：获得性血友病A（Acquired hemophilia A, AHA）是因机体产生针对凝血因子VIII（coagulation factor VIII, FVIII）的自身抗体，导致FVIII活性显著缺乏，进而引发可危及生命的严重出血症状，因此AHA患者往往需要接受输血治疗。艾美赛珠单抗（emicizumab）是一种可模拟活化FVIII功能的双特异性单克隆抗体（bispecific monoclonal antibody），目前已被更广泛地应用于AHA的临床治疗。
病例报告：本文报告1例70岁男性耶和华见证人（Jehovah's Witness）AHA患者，该患者左肱二头肌出现严重出血，且贫血在短短2天内进展约100×10^4/μL，但拒绝接受输血治疗。鉴于凝血因子类药物的使用已获得书面批准，我们为其给予单剂量活化凝血因子VIIa/X（activated factor VIIa/X, Byclot®）混合制剂，迅速实现了止血。后续定期给予艾美赛珠单抗治疗，有效预防了再出血，并成功避免了输血。
结论：对于合并AHA的耶和华见证人患者，艾美赛珠单抗可被视为极具临床价值的治疗方案。