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B 结构域缺失的重组凝血因子 VIII 持续输注用于严重 A 型血友病患者骨科手术:首例病例报告。

Continuous infusions of B domain-truncated recombinant factor VIII, turoctocog alfa, for orthopedic surgery in severe hemophilia A: first case report.

机构信息

Department of Pediatrics, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, 634-8522, Japan.

Department of Orthopedic Surgery, Nara Medical University, Kashihara, Nara, Japan.

出版信息

Int J Hematol. 2018 Aug;108(2):199-202. doi: 10.1007/s12185-018-2415-5. Epub 2018 Jan 30.

Abstract

Continuous infusions (CI) of factor (F)VIII are preferable to the conventional bolus injections for the maintenance of consistent FVIII levels during surgery in patients with severe hemophilia A. A third generation, B domain-truncated recombinant FVIII (turoctocog alfa, Novo Nordisk, NovoEight), was approved for clinical use in 2014. The hemostatic efficacy and safety of bolus injections of turoctocog alfa in patients undergoing surgery have been reported, but no reports on CI therapy have been published. We describe a 43-year-old patient with severe hemophilia A who required arthroscopic synovectomy of the right elbow and arthrodesis of the right ankle. He was treated with a bolus injection of turoctocog alfa (36 IU/kg) immediately before operation, followed by CI (infusion rate; 2.9 IU/kg/h) to maintain FVIII activity > 80 IU/dl throughout the perioperative period. Surgery was completed successfully with uncomplicated hemostatic control. CIs were continued until post-operative day (POD) 4. Further bolus injections were given from POD5. No anti-FVIII inhibitor has been detected post-operation. This case provides important information on CI therapy using turoctocog alfa during surgery for patients with severe hemophilia A.

摘要

在严重 A 型血友病患者的手术中,持续输注因子(FVIII)优于常规推注,因为它可以在手术期间保持稳定的 FVIII 水平。第三代 B 结构域缺失的重组 FVIII(turoctocog alfa,诺和诺德,NovoEight)于 2014 年获准临床使用。已经报道了用于手术患者的 turoctocog alfa 推注的止血效果和安全性,但没有关于 CI 治疗的报告。我们描述了一位 43 岁的严重 A 型血友病患者,他需要进行右肘关节镜滑膜切除术和右踝关节融合术。他在手术前立即接受了 turoctocog alfa(36 IU/kg)的推注治疗,然后进行 CI(输注率;2.9 IU/kg/h),以在整个围手术期维持 FVIII 活性>80 IU/dl。手术顺利完成,止血控制良好。CI 持续到术后第 4 天(POD4)。从 POD5 开始进一步推注。手术后未检测到抗 FVIII 抑制剂。该病例提供了有关严重 A 型血友病患者手术期间使用 turoctocog alfa 进行 CI 治疗的重要信息。

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