Totonchi Ali, Eshraghi Yashar, Beck Daniel, McCrae Keith, Guyuron Bahman
Department of Plastic and Reconstructive Surgery, Case Western Reserve University, Cleveland, Ohio, USA.
Aesthet Surg J. 2008 Mar-Apr;28(2):189-94. doi: 10.1016/j.asj.2007.12.002.
Von Willebrand disease (vWD), a hemorrhagic disorder mimicking a defect in platelet function, is the most commonly inherited coagulopathy, resulting in a deficiency that may prolong bleeding time and increase risk for major bleeding complications during surgery. Von Willebrand factor (vWF) serves a dual role in hemostasis: mediating the initial platelet adhesion to damaged endothelium at the site of vessel injury and stabilizing coagulation factor VIII, an important cofactor in the generation of a fibrin clot. Although quantitative or qualitative defects in vWF protein can manifest as a mild to severe bleeding disorder, many cases of vWD remain subclinical, barring major invasive stimuli, and undetected by either patient or clinician. Nevertheless, the frequency of this coagulation disorder would almost ensure that every plastic surgeon will encounter affected patients, making a thorough understanding of vWD and its management absolutely necessary. Surprisingly, there is little information concerning vWD in the plastic surgery literature. Our goal is to familiarize the plastic surgeon with vWD, including physiology, diagnostic criteria, classification, and molecular basis for multiple vWD variants, and diagnosis and management.
血管性血友病（vWD）是一种类似血小板功能缺陷的出血性疾病，是最常见的遗传性凝血病，其导致的缺陷可能会延长出血时间，并增加手术期间发生大出血并发症的风险。血管性血友病因子（vWF）在止血过程中发挥双重作用：在血管损伤部位介导血小板与受损内皮的初始黏附，并稳定凝血因子VIII，后者是纤维蛋白凝块形成过程中的一种重要辅助因子。尽管vWF蛋白的定量或定性缺陷可表现为轻度至重度出血性疾病，但许多vWD病例仍处于亚临床状态，除非受到重大侵入性刺激，患者和临床医生均无法检测到。然而，这种凝血病的发病率几乎可以肯定，每位整形外科医生都会遇到受影响的患者，因此全面了解vWD及其治疗方法绝对必要。令人惊讶的是，整形外科文献中关于vWD的信息很少。我们的目标是让整形外科医生熟悉vWD，包括其生理学、诊断标准、分类、多种vWD变体的分子基础以及诊断和治疗。
