A Jehovah's Witness with Acquired Hemophilia A Who Successfully Avoided Blood Transfusions by Receiving Emicizumab.
Аннотация
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 3 g/dL (hemoglobin level) 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.
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