[Von Willebrand disease: a common and unrecognized bleeding disorder]

The von Willebrand factor plays an important role in primary and secondary hemostasis. Von Willebrand disease is due to a quantitative or qualitative abnormality of von Willebrand factor. It is the most frequent constitutional abnormality of hemostasis. The main manifestation of von Willebrand disease is a mucocutaneous hemorrhagic syndrome; however, von Willebrand disease is characterized by an important clinical and biological heterogeneity. Its transmission is autosomal, usually dominant. A screening can be made by a general practitioner on the basis of a good clinical history. It is then important to confirm the diagnosis in a specialized laboratory because an adequate treatment can be prescribed to avoid hemorrhagic complications, in particular at the time of surgical procedures.