Alteraciones del sistema hemostático

Hemostasis is a dynamic process that involves the finely coordinated regulation of various components, including platelets, plasma proteins, and the endothelium, to maintain hemostatic balance and prevent bleeding diathesis and thrombosis. The understanding of this process has evolved toward a model where interactions occur mainly on cell surfaces. This update analyzes the physiology of hemostasis, with greater emphasis on secondary hemostasis. It addresses various diagnostic techniques used to evaluate bleeding diathesis, from basic studies to molecular analysis, highlighting the importance of molecular diagnostics in the accurate identification of these disorders. A review of the pathophysiology, diagnosis, and treatment of the most common congenital coagulation disorders (hemophilia and von Willebrand disease) is also presented, along with a brief mention of less common hereditary coagulopathies and congenital hyperfibrinolysis.