VON WILLEBRAND DISEASE

Abstract

Described by Erik Adolf von Willebrand in 1926, it is a constitutional hemorrhagic disease, inherited in the autosomal mode. Considered initially as a thrombopathic disease, then as a prologation of the bleeding time associated to a defi cit in the factor VIII, today it is defi ned as an absence, diminuation or a modifi cation of the von Willebrand factor, a glycoprotein sintetized by the endotheklial cell and the megariocyte. The incidence of the disease is around 2%, being the fi rst constitutionally hemorrhagic defi cit, in front of the hemophilias. In this article we present: phisiopathologic mechanisms of the disease, clinical signs, biologic diagnosis, classifi cation of von Willebrand disease, the differential diagnosis and treatment

Keywords

• von willebrand disease
• child