Division of Cardiovascular Health and Disease, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Stonehill Professor of Medicine, University of Cincinnati College of Medicine, 231 Albert Sabin Way, CVC 4th Floor, Room 4936, Cincinnati, 45267, OH, USA.
J Thromb Thrombolysis. 2019 Jul;48(1):14-26. doi: 10.1007/s11239-019-01849-2.
Von Willebrand Factor (vWF) is a large glycoprotein with a broad range of physiological and pathological functions in health and disease. While vWF is critical for normal hemostasis, vascular integrity and repair, quantitative and qualitative abnormalities in the molecule can predispose to serious bleeding and thrombosis. The heritable form of von Willebrand Disease was first described nearly a century ago, but more recently, recognition of an acquired condition known as acquired von Willebrand Syndrome (AVWF) has emerged in persons with hematological, endocrine and cardiovascular diseases, disorders and conditions. An in-depth understanding of the causes, diagnostic approach and management of AVWS is important for practicing clinicians.
血管性血友病因子(vWF)是一种大型糖蛋白,在健康和疾病中具有广泛的生理和病理功能。虽然 vWF 对正常止血、血管完整性和修复至关重要,但该分子的数量和质量异常可导致严重出血和血栓形成。遗传性血管性血友病的形式在近一个世纪前就已被描述,但最近,在患有血液学、内分泌和心血管疾病、疾病和病症的人群中,人们认识到一种称为获得性血管性血友病综合征(AVWS)的获得性疾病。深入了解 AVWS 的病因、诊断方法和治疗对于临床医生非常重要。