[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.
Boehlen
F
F
Unité d'hémostase, HUG, Genève. francoise.boehlen@hcuge.ch
Robert-Ebadi
H
H
de Moerloose
P
P
D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D014842:von Willebrand Diseases / Q000097:blood / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics; D014841:von Willebrand Factor / Q000378:metabolism
D014841:von Willebrand Factor