Bleeding in the GI tract continues to pose a therapeutic challenge for clinicians in patients with Von Willebrand Disease (VWD). It is associated with significant morbidity and mortality and represents the major unmet need in VWD. Defective angiogenesis in the gut is primarily responsible, resulting in angiodysplastic malformations making bleeding notoriously refractory to standard replacement therapy. A substantial body of evidence now shows that Von Willebrand Factor (VWF) has a role in the regulation of angiogenesis but the mechanisms responsible for the formation of vascular malformations remain incompletely understood. Data from the wider field of vascular malformations may lend insight and point to novel therapeutic approaches. Here we review evidence linking VWF to angiodysplasia, the associated molecular mechanisms and the implications for therapy.

中文翻译：

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血管性血友病和血管发育不良：寻求治疗的发病机制的更广泛视角。


胃肠道出血继续对血管性血友病 （VWD） 患者的临床医生构成治疗挑战。它与显著的并发症发生率和死亡率相关，代表了 VWD 中未满足的主要需求。肠道血管生成缺陷是主要原因，导致血管发育不良畸形，使出血对标准替代疗法难以治疗。现在大量证据表明，血管性血友病因子 （VWF） 在血管生成的调节中发挥作用，但导致血管畸形形成的机制仍不完全清楚。来自更广泛血管畸形领域的数据可能会提供见解并指出新的治疗方法。在这里，我们回顾了将 VWF 与血管发育不良联系起来的证据、相关的分子机制和对治疗的影响。