Abstract

Abnormalities in the hemostatic system: Hemostasis is a complex process that a multitude of agents (endothelium, proteins, platelets, etc.) participate in in order to achieve a state of homeostasis, avoid spontaneous hemorrhage, and limit bleeding when a lesion occurs. Knowledge has been progressing towards a model in which coagulation reactions occur on cell surfaces. Congenital coagulopathies are classified as mild, moderate, or severe based on the factor deficiency. We will study hemophilias A and B, von Willebrand disease, and other less frequent coagulopathies due to deficits of other factors based on the medical history of hemorrhage and determination of the coagulation factor level. The study of von Willebrand disease will be among the most complex. The molecular study of these disorders is gaining great importance, given that it can help with diagnosis, predict the risk of developing inhibitors (hemophilias), and will begin allowing for establishing a correlation between the hemorrhagic genotype and phenotype. Treatment is based on replacement of the deficient factor. It is recommended to do so using the available commercial formulas, individualizing treatments by making the patient a participant in them, and evaluating other adjuvant treatments to favor the hemostatic process.

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