Maintaining normal hemostasis relies on a regulated system of procoagulant and anticoagulant pathways, and disruption of these processes leads to the loss of hemostatic control, with the potential for excessive bleeding or thrombosis. Evaluation of bleeding disorders has conventionally been achieved by laboratory assays that measure the activity of individual coagulation factors. While such assays have proven effective for detecting abnormalities of the coagulation system and aiding diagnosis, inherent limitations prevent them from capturing a complete picture of hemostatic function. An improved understanding of thrombin activity and its central role in hemostasis and bleeding disorders has led to the clinical development of global assays that are more physiologically relevant than traditional assays; furthermore, these global assays are able to monitor responses to therapy. In this review, we provide an overview of the role of thrombin in hemostasis, and describe the clinical benefits of thrombin monitoring in patients with bleeding disorders. Moreover, we discuss recent advances in thrombin-targeting therapeutic strategies that aim to correct thrombin deficiency and prevent bleeding in patients with hemophilia and other rare bleeding disorders.

维持正常的止血依赖于促凝和抗凝途径的调节系统，这些过程的破坏会导致止血控制的丧失，并可能导致过多的出血或血栓形成。常规上，通过测量单个凝血因子活性的实验室测定已经实现了对出血性疾病的评估。尽管已证明此类测定法可有效检测凝血系统异常并帮助诊断，但固有的局限性阻止了它们获得止血功能的完整图像。对凝血酶活性及其在止血和出血性疾病中的重要作用的更好的理解导致了与传统方法相比在生理上更相关的整体方法的临床发展。此外，这些整体测定法能够监测对治疗的反应。在这篇综述中，我们概述了凝血酶在止血中的作用，并描述了凝血酶监测对出血性疾病患者的临床益处。此外，我们讨论了针对凝血酶的治疗策略的最新进展，这些策略旨在纠正凝血酶缺乏症并预防血友病和其他罕见出血性疾病患者的出血。