Alteraciones del sistema hemostático. Estrategias diagnósticas de la patología hemorrágica. Coagulopatías congénitas

Abstract

IntroductionHemostasis is a complex mechanism of multiple interactions between cells, vascular endothelium and plasma proteins, allowing proper blood circulation. It allows blood to flow without forming thrombosis in the vessels and also prevent bleeding when damage occurs. Among these mechanisms, the coagulation system is the most important of the hemostatic process, which ends with the formation of insoluble fibrin, the main component of the clot. EtiologyCongenital coagulation disorders are hereditary defects caused by genetic alterations in procoagulant clotting factors. The most common are the von Willebrand disease and hemophilia A and B. Diagnosis and treatmentThe diagnosis in hemophilia is simple, but its management is complicated and treatment has several complications. However, in von Willebrand disease diagnosis, is the most complicated thing. Other congenital coagulopathies called «rare» include defects in any of the other coagulation factors: fibrinogen, factor XIII, factor XI, factor X, factor VII, factor V and factor I. Its low incidence is a problem. It is necessary to unify experiences to establish a proper diagnosis and treatment.