Ogawa Yoshihiko, Nishida Yasuharu, Kyo Takayuki, Miki Hidenobu, Yonemoto Hitoshi, Bandou Hiroki, Yajima Keishiro, Kasai Daisuke, Taniguchi Tomohiro, Watanabe Dai, Uehira Tomoko, Ueda Takahumi, Shirasaka Takuma
Department of Infectious Disease, Osaka National Hospital.
Rinsho Ketsueki. 2011 Aug;52(8):713-7.
Major surgery in hemophilia patients has been facilitated by the development of coagulation concentrates. However, it is still difficult to manage bleeding during major surgery in patients with inhibitors to FVIII/IX. In addition, there have been few reports of major surgery in hemophilia B with high responding inhibitors. We report a 26-year-old hemophilia B patient with high responding factor IX inhibitor who demonstrated severe hemophiliac arthropathy in his left hip joint. Total hip arthroplasty was performed with a high dose of FIX followed by recombinant FVIIa. His inhibitor titer was decreased from 111 BU/ml to 1.0 BU/ml at surgery by avoiding the use of FIX concentrates. Thus, we could use high dose FIX for the management of surgical bleeding. Anamnestic response occurred on the 7th day after surgery and FIX concentrates were switched to recombinant FVIIa. The whole process was safely managed without any excess bleeding or adverse effects. The successful use of high dose FIX followed by recombinant FVIIa suggests that even major surgery could be safely performed in hemophilia B patients with a low titer of high responding inhibitors.
凝血因子浓缩剂的发展推动了血友病患者的大手术开展。然而，对于FVIII/IX抑制剂患者，大手术期间的出血管理仍很困难。此外，关于高反应性抑制剂的B型血友病患者进行大手术的报道很少。我们报告了一名26岁的B型血友病患者，其因子IX抑制剂反应高，左髋关节出现严重血友病性关节病。在高剂量FIX随后联合重组FVIIa的情况下进行了全髋关节置换术。通过避免使用FIX浓缩剂，其抑制剂滴度在手术时从111 BU/ml降至1.0 BU/ml。因此，我们能够使用高剂量FIX来处理手术出血。术后第7天出现了回忆反应，于是将FIX浓缩剂换为重组FVIIa。整个过程安全管理，未出现任何过度出血或不良反应。高剂量FIX随后联合重组FVIIa的成功应用表明，即使是低滴度高反应性抑制剂的B型血友病患者也能安全地进行大手术。
