Low von Willebrand factor: sometimes a risk factor and sometimes a disease.
Low von Willebrand factor: sometimes a risk factor and sometimes a disease.
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DOI：
10.1182/asheducation-2009.1.106
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发表时间：
2009-01-01
期刊：
影响因子：
--
通讯作者：
Sadler, J Evan
中科院分区：
文献类型：
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作者：
Sadler, J Evan
血管性血友病因子（VWF）水平足够低易导致相当严重的出血，低VWF是1型血管性血友病（VWD）的诊断特征，其特征是VWF的部分定量缺乏。最近对1型VWD患者的突破性研究已经描述了几种决定VWF血浆浓度的病理生理机制，但VWF水平与出血可能性之间的关系仍不太清楚。在某种程度上，这个问题反映了人群中VWF水平的广泛范围，因此“正常”和“低”之间的区别是任意的。出血的风险当然会随着VWF水平的降低而增加，但在VWF水平非常低之前，这种关系并不是很强。此外，轻度出血症状在明显健康的人群中很常见，并且除了VWF缺陷之外还有许多原因，这使得不可能将出血归因于任何单一因素，例如低VWF。这些困难可能通过流行病学方法来解决VWF和其他出血风险因素，类似于医生如何管理心血管疾病或静脉血栓栓塞的多个风险因素。
A sufficiently low level of von Willebrand factor (VWF) predisposes to bleeding that can be quite serious, and low VWF is a diagnostic feature of von Willebrand disease (VWD) type 1, which is characterized by partial quantitative deficiency of VWF. Recent groundbreaking studies of patients with VWD type 1 have delineated several pathophysiologic mechanisms that determine the plasma concentration of VWF, but the relationship between VWF level and the likelihood of bleeding remains less well understood. In part, this problem reflects the broad range of VWF levels in the population, so that the distinction between "normal" and "low" is arbitrary. The risk of bleeding certainly increases as the VWF level decreases, but the relationship is not very strong until the VWF level is very low. Furthermore, mild bleeding symptoms are common in apparently healthy populations and have many causes other than defects in VWF, which can make it impossible to attribute bleeding to any single factor, such as low VWF. These difficulties might be resolved by an epidemiologic approach to VWF and other risk factors for bleeding, analogous to how physicians manage multiple risk factors for cardiovascular disease or venous thromboembolism.