Department of Rheumatology and Nephrology, Hokkaido University Hospital, Sapporo, Japan.
Department of Rheumatology, Nephrology and Endocrinology, Faculty of Medicine, Hokkaido University, Kita 14, Nishi 5, Kita-Ku, Sapporo, 060-8648, Japan.
CEN Case Rep. 2024 Apr;13(2):117-120. doi: 10.1007/s13730-023-00811-9. Epub 2023 Jul 25.
We report the first case of hemophilia A with factor VIII (FVIII) inhibitor who received hemodialysis via an arteriovenous (AV) fistula. Hemophilia A is a congenital deficiency of blood coagulation FVIII that is characterized by prolonged bleeding. Approximately 30% of patients with hemophilia develop allogeneic antibodies of FVIII. The inhibitors decrease the hemostatic effect of replacement therapy; thus, the prophylaxis strategy should be well designed. Prophylactic treatment with invasive procedures is needed to prevent excessive bleeding in patients with hemophilia undergoing hemodialysis. On the contrary, hemodialysis requires attention to the development of intracircuit coagulation during dialysis. Peritoneal dialysis or hemodialysis with a long-term tunneled central venous catheter has mainly been selected as the dialysis modality for patients with hemophilia and end-stage renal disease requiring renal replacement therapy because hemodialysis with an arteriovenous fistula may result in bleeding from the puncture site after each hemodialysis session. In our patient, hemodialysis was safely performed without any anticoagulant agents, and replacement therapy with FVIII concentrates prevented bleeding after puncture of the AV fistula.
我们报告了首例接受动静脉（AV）瘘管血液透析的因子 VIII（FVIII）抑制剂血友病 A 患者。血友病 A 是一种先天性凝血因子 FVIII 缺乏症，其特征为出血时间延长。约 30%的血友病患者会产生 FVIII 的同种异体抗体。抑制剂会降低替代治疗的止血效果；因此，应精心设计预防策略。对于接受血液透析的血友病患者，需要通过有创操作进行预防性治疗，以防止过度出血。相反，血液透析需要注意在透析过程中回路内凝血的发展。对于需要肾替代治疗的血友病和终末期肾病患者，主要选择腹膜透析或带长期隧道中心静脉导管的血液透析作为透析方式，因为动静脉瘘血液透析可能会导致每次血液透析后穿刺部位出血。在我们的患者中，无需任何抗凝剂即可安全地进行血液透析，FVIII 浓缩物的替代治疗可防止 AV 瘘管穿刺后的出血。
