Tran-Thang C, Mannucci P M, Schneider P, Federici A, Bachmann F
Br J Haematol. 1985 Oct;61(2):307-14. doi: 10.1111/j.1365-2141.1985.tb02830.x.
Laboratory investigation of an acquired haemorrhagic diathesis in a 63-year-old man with malignant lymphoma revealed the classical haemostatic defects found in von Willebrand's disease (vWD). In addition, SDS-agarose gel electrophoresis demonstrated alterations of the von Willebrand factor (vWF) multimeric structure. A profound defect of large and intermediate size multimers was observed which was different from those seen in variants of congenital vWD. In vitro, weak inhibitory activity against factor VIII procoagulant activity and ristocetin cofactor activity was present in the patient's plasma. When patient's plasma was incubated with normal plasma, followed by centrifugation, vWF antigen (vWF:Ag) was precipitated. In vivo, after transfusion of cryoprecipitate, there was rapid plasma clearance of vWF:Ag and ristocetin cofactor and of FVIII coagulant activities.
对一名患有恶性淋巴瘤的63岁男性获得性出血素质进行的实验室检查显示，其具有血管性血友病（vWD）中典型的止血缺陷。此外，十二烷基硫酸钠-琼脂糖凝胶电泳显示血管性血友病因子（vWF）多聚体结构发生改变。观察到大小和中等大小多聚体存在严重缺陷，这与先天性vWD变体中所见不同。在体外，患者血浆中存在对凝血因子VIII促凝活性和瑞斯托霉素辅因子活性的微弱抑制活性。当患者血浆与正常血浆孵育，随后离心时，vWF抗原（vWF:Ag）沉淀。在体内，输注冷沉淀后，vWF:Ag、瑞斯托霉素辅因子和FVIII凝血活性的血浆清除迅速。
