Institute of Medicine, Tribhuvan University, Maharajgunj 44600, Nepal.
Isfahan Neuroscience Research Center, Isfahan, Iran.
Int J Clin Pract. 2022 Aug 27;2022:5369001. doi: 10.1155/2022/5369001. eCollection 2022.
Bleeding disorders are a major group of hematological disorders, which are highly prevalent in the world. Excessive bleeding can result in serious consequences including hypoperfusion and cardiac arrest. The body has its selfmechanism to control excessive bleeding which is termed hemostasis. Hemostasis is achieved in two major steps, the formation of the primary and secondary hemostatic plugs. Endothelium, platelets, and coagulation factors are three components involved in hemostasis. Endothelium and platelets have a major role in forming the primary hemostatic plug. Consequently, the first step in investigating a bleeding disorder is platelet count. Despite normal platelet count, abnormality in the primary hemostatic plug may arise due to functional defects of the platelets including adhesion, activation, and aggregation. Von Willebrand disease (VWD) is an endothelial defect and the most prevalent inherited defect in coagulation. Abnormalities in the secondary hemostatic plug are largely due to coagulation factor deficiencies, and, to a lesser extent, the presence of inhibitors. Techniques involving viscoelastics have been aiding in rapid diagnosis and are useful in point-of-care testing. This article discusses the investigation of bleeding disorders from the perspective of the endothelium, platelet, and coagulation factor physiology. These three components should be properly investigated to achieve the definitive diagnosis of bleeding disorders.
出血性疾病是一组主要的血液系统疾病,在世界范围内发病率很高。过度出血会导致严重后果,包括灌注不足和心脏骤停。人体有自己的机制来控制过度出血,称为止血。止血分为两个主要步骤,即初级和次级止血栓的形成。内皮细胞、血小板和凝血因子是止血过程中涉及的三个组成部分。内皮细胞和血小板在形成初级止血栓中起主要作用。因此,调查出血性疾病的第一步是血小板计数。尽管血小板计数正常,但由于血小板的功能缺陷,包括粘附、激活和聚集,初级止血栓可能出现异常。血管性血友病(VWD)是一种内皮细胞缺陷,也是最常见的遗传性凝血缺陷。次级止血栓的异常主要归因于凝血因子缺乏,在较小程度上归因于抑制剂的存在。涉及粘弹性的技术一直在辅助快速诊断,并在即时护理测试中很有用。本文从内皮细胞、血小板和凝血因子生理学的角度讨论了出血性疾病的研究。为了明确诊断出血性疾病,应适当检查这三个组成部分。