Enfermedades de la hemostasia primaria

Abstract

This update focuses on disorders that affect the vascular endothelium and platelets, key components in primary hemostasis. These are classified into purpura, which involves vascular abnormalities, and quantitative or qualitative platelet abnormalities, both of which can be congenital or acquired. The most common hemorrhagic symptoms include cutaneous and mucosal bleeding following trauma or surgery. Some congenital coagulation disorders are part of more complex syndromes that go beyond hemorrhagic symptoms and require a multidisciplinary approach. It is crucial to take a detailed medical history and perform a thorough physical examination when faced with any coagulation disorder. Platelet function tests and molecular diagnostic techniques are important additional tools. Regarding treatment, bleeding complications are treated in many cases according to their severity with desmopressin, antifibrinolytic agents, platelet transfusion, recombinant activated factor VII, and thrombopoietin receptor agonists. In some cases of congenital origin, allogeneic transplantation of hematopoietic progenitors is the only curative option. In acquired forms, treatment will generally be for the underlying disease itself.