Young P H, Bouhasin J D, Barner H B
J Thorac Cardiovasc Surg. 1978 Aug;76(2):218-22.
A patient with von Willebrand's disease having aortic valve replacement was managed with cryoprecipitate infusions and monitoring of factor VIII levels. This disorder is associated with low factor VIII levels and abnormal platelet function. There may be no history of bleeding, as the severity of the bleeding tendency varies greatly and fluctuates temporally. The partial thromboplastin time is frequently prolonged, but more detailed studies are necessary to establish a diagnosis (bleeding time, platelet adhesiveness to glass beads and ristocetin, von Willebrand's antigen, ristocetin-von Willebrand's factor, and factor VIII clotting activity). Elevation of factor VIII levels to 50 to 100% of normal allows adequate clotting and is best accomplished with cryoprecipitate or fresh frozen plasma rather than commercial concentrates of factor VIII, whose activity is unpredictable.
一名患有血管性血友病的患者接受主动脉瓣置换手术，术中通过输注冷沉淀并监测因子VIII水平进行处理。这种疾病与因子VIII水平降低和血小板功能异常有关。可能没有出血史，因为出血倾向的严重程度差异很大且随时间波动。部分凝血活酶时间常常延长，但需要更详细的检查来确诊（出血时间、血小板对玻璃珠和瑞斯托霉素的黏附性、血管性血友病抗原、瑞斯托霉素-血管性血友病因子以及因子VIII凝血活性）。将因子VIII水平提高至正常的50%至100%可实现充分凝血，最好使用冷沉淀或新鲜冰冻血浆，而非活性不可预测的因子VIII商业浓缩剂。
