Doença de von Willebrand: características, diagnóstico laboratorial e tratamento

Abstract

Von Willebrand Disease (vWD) is a hereditary coagulopathy with high prevalence worldwide, characterized by qualitative or quantitative mutations in the von Willebrand factor. These mutations, in turn, lead to bleeding episodes and symptoms that vary in severity according to the mutation causing the disease. vWD is classified into types 1, 2, and 3, with type 2 further divided into four subtypes, each requiring specific examinations for proper differential diagnosis and treatment in accordance with the alterations they produce. With this in mind, this work reviews articles from the last decade with the aim of presenting the most recent guidelines on the diagnosis and treatment of vWD, along with situations and conditions that may affect both, such as pregnancy and aging. This study highlights not only the reference values of the main vWD variants and diagnostic tests used for the disease, but also the greater efficacy of prophylactic treatment compared to on-demand approaches in reducing the frequency and intensity of bleeding episodes. [resumo fornecido pelo autor]