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<p>Acquired hemophilia A (AHA) is a rare autoimmune disorder characterized by coagulation factor VIII inhibitors, leading to severe bleeding. Patients often present with bleeding after invasive procedures, necessitating prompt diagnosis and management. Here, we report the case of an 83-year-old man diagnosed with AHA after experiencing persistent bleeding from a hemodialysis catheter insertion site. The catheter was placed for acute kidney injury (AKI) associated with nephrotic syndrome. Despite prednisolone therapy for nephrotic syndrome, he developed factor VIII inhibitors and significant bleeding, suggesting insufficient immunosuppression. Given his advanced age, renal failure, and recurrent pneumonia, intensified immunosuppressive therapy posed a high risk of infection. Therefore, a hemostasis-centered approach was prioritized. Recombinant activated factor VII (rFVIIa, eptacog alfa) failed to control the bleeding; however, recombinant porcine sequence factor VIII (rpFVIII, susoctocog alfa) proved effective, enabling the safe placement of a long-term indwelling catheter for hemodialysis. To maintain hemostasis, emicizumab was introduced, allowing outpatient hemodialysis without further bleeding. Reports of AHA in hemodialysis patients are scarce. To the best of our knowledge, there have been no prior reports of AHA cases requiring hemodialysis that were successfully treated with susoctocog alfa and emicizumab. This case highlights the potential role of these agents in optimizing hemostatic strategies for AHA patients undergoing hemodialysis.</p>

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Successful hemodialysis initiation in acquired hemophilia a managed with susoctocog alfa and emicizumab

  • 期刊论文

  • 发表日期：

    2025年12月24日

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  • Ayano Kihara,
  • Masanori Tamaki,
  • Ryohei Sumitani,
  • Chihiro Okinari,
  • Miho Tada,
  • Shinji Miyakami,
  • Sumiyo Yamaguchi,
  • Taizo Inagaki,
  • Masanori Minato,
  • Eriko Shibata,
  • Kazuhiro Hasegawa,
  • Shu Wakino

摘要

Acquired hemophilia A (AHA) is a rare autoimmune disorder characterized by coagulation factor VIII inhibitors, leading to severe bleeding. Patients often present with bleeding after invasive procedures, necessitating prompt diagnosis and management. Here, we report the case of an 83-year-old man diagnosed with AHA after experiencing persistent bleeding from a hemodialysis catheter insertion site. The catheter was placed for acute kidney injury (AKI) associated with nephrotic syndrome. Despite prednisolone therapy for nephrotic syndrome, he developed factor VIII inhibitors and significant bleeding, suggesting insufficient immunosuppression. Given his advanced age, renal failure, and recurrent pneumonia, intensified immunosuppressive therapy posed a high risk of infection. Therefore, a hemostasis-centered approach was prioritized. Recombinant activated factor VII (rFVIIa, eptacog alfa) failed to control the bleeding; however, recombinant porcine sequence factor VIII (rpFVIII, susoctocog alfa) proved effective, enabling the safe placement of a long-term indwelling catheter for hemodialysis. To maintain hemostasis, emicizumab was introduced, allowing outpatient hemodialysis without further bleeding. Reports of AHA in hemodialysis patients are scarce. To the best of our knowledge, there have been no prior reports of AHA cases requiring hemodialysis that were successfully treated with susoctocog alfa and emicizumab. This case highlights the potential role of these agents in optimizing hemostatic strategies for AHA patients undergoing hemodialysis.