Mohri Hiroshi
Division of Health Promotion Service, Department of Community and Environmental Health, Tokai University School of Medicine, Bousei-Dai, Isehara, Kanagawa 259-1193, Japan.
J Thromb Thrombolysis. 2003 Jun;15(3):141-9. doi: 10.1023/B:THRO.0000011369.70824.e6.
Acquired von Willebrand syndrome is a bleeding disorder associated with various underlying diseases. The clinical manifestations are similar to congenital von Willebrand disease. Diagnosis is mainly confirmed by decrease of ristocetin cofactor activity (vWF:RCo) and collagen binding activity (vWF:CBA) and by vWF multimeric analysis usually with selective loss of large multimers. Various pathogenic mechanisms have been proposed, including development of autoantibodies to von Willebrand factor (vWF), adsorption of vWF onto tumor cells or activated platelets, increase of vWF proteolysis, and mechanical destruction of vWF under high shear stress. Following the treatment of underlying disorders, desmopressin (DDAVP) is a first intention therapeutic option. Factor VIII/vWF concentrates and high dose immunoglobulin infusions are reserved for patients unresponsive to DDAVP.
获得性血管性血友病综合征是一种与多种基础疾病相关的出血性疾病。其临床表现与先天性血管性血友病相似。诊断主要通过瑞斯托霉素辅因子活性（vWF:RCo）和胶原结合活性（vWF:CBA）降低以及vWF多聚体分析来确诊，通常表现为大的多聚体选择性丢失。已提出多种致病机制，包括针对血管性血友病因子（vWF）的自身抗体形成、vWF吸附到肿瘤细胞或活化血小板上、vWF蛋白水解增加以及在高剪切应力下vWF的机械破坏。在治疗基础疾病后，去氨加压素（DDAVP）是首选治疗方案。VIII因子/vWF浓缩物和高剂量免疫球蛋白输注适用于对DDAVP无反应的患者。
