Komada Tetsuya, Kawasaki Takashi, Sata Takeyoshi
Department of Anesthesiology, University of Occupational and Environmental Health, Kitakyushu 807-8555.
Masui. 2013 Nov;62(11):1368-71.
Acquired von Willebrand syndrome (AvWS) is generic name of bleeding disorders caused by acquired decrease of von Willebrand factor. A 25-year-old woman with AvWS underwent emergent cesarean section under combined spinal and epidural anesthesia. Before operation, her APTT was within normal ranges. Platelet count was about 80,000 and factor VIII and von Willebrand factor's activities were 37% and 21%, respectively. Therefore, we administered 10 units of platelet concentrate and 2,000 units of factor VIII concentrate intravenously before the operation. The emergent operation was done without any bleeding complications. Factor VIII concentrate was administered until 11 days after the operation. Epidural catheter was removed just after administration of factor VIII concentrate 3 days after operation. The perioperative course was uneventful and the patient was discharged 11 days after the operation. It is important to co-operate with other related departments in managing the perioperative coagulation status in a patient with AvWS.
获得性血管性血友病综合征（AvWS）是由血管性血友病因子获得性减少引起的出血性疾病的统称。一名患有AvWS的25岁女性在腰麻联合硬膜外麻醉下接受了急诊剖宫产手术。术前，她的活化部分凝血活酶时间（APTT）在正常范围内。血小板计数约为80,000，凝血因子VIII和血管性血友病因子的活性分别为37%和21%。因此，我们在手术前静脉输注了10单位血小板浓缩物和2000单位凝血因子VIII浓缩物。急诊手术顺利完成，未出现任何出血并发症。术后持续给予凝血因子VIII浓缩物直至术后11天。术后3天在输注凝血因子VIII浓缩物后立即拔除硬膜外导管。围手术期过程顺利，患者术后11天出院。在管理AvWS患者的围手术期凝血状态时，与其他相关科室合作很重要。
