Abstract

Diseases of primary hemostasis. Vascular purpura. Platelet disorders: This update addresses the group of disorders that affects blood vessel walls and platelets, which are a fundamental part of primary hemostasis. They are divided into purpura (vascular involvement) or quantitative or qualitative platelet abnormalities and can be congenital or acquired. The incidence of many of these entities is low; some are considered rare diseases. The typical hemorrhagic manifestations include the appearance of cutaneous and mucosal bleeding with trauma. Some congenital coagulopathies form part of groups of syndromes in which involvement goes beyond bleeding symptoms, requiring a multidisciplinary approach. In all coagulopathies, it is important to take a thorough personal and family medical history. In this regard, platelet function and aggregation tests are useful. More and more, the genetic alterations behind these cases are becoming known, with molecular diagnostic techniques gaining greater importance. In congenital forms, treatment is symptomatic, targeted according to the localization, and accompanied by multidisciplinary teams. For cases with platelet function involvement, a platelet transfusion can also be opted for.

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