Willebrand disease mainly results in hemorrhages from the nose, gums, vagina during menstruation, etc.
- Willebrand disease is a hereditary hemorrhagic pathology due to a genetic defect in the concentration, structure or function of Willebrand Factor (VWF), a protein involved in the mechanisms of primary hemostasis and coagulation.
- In its most common form, Willebrand disease mainly results in hemorrhages.
- In its moderate form, Willebrand disease is considered the most common of familial hemorrhagic diseases, its prevalence being estimated between 0.5 and 1.1%.
On the occasion of World Bleeding Awareness Day this Wednesday, April 17, the MHEMO (Health sector for constitutional hemorrhagic diseases) would like to draw attention to Willebrand disease (WD).
“Willebrand disease is a hereditary hemorrhagic pathology due to a genetic defect in the concentration, structure or function of Willebrand Factor (VWF), a protein involved in the mechanisms of primary hemostasis and coagulation,” first explains the association.
Willebrand disease: what are the symptoms?
There are several types of Willebrand disease. In its most common form, this pathology essentially results in hemorrhages:
· nosebleeds or epistaxis;
· bleeding gums or gingival bleeding;
· bruises or bruising;
· prolonged bleeding from minor skin injuries;
· heavy periods or menorrhagia;
· digestive bleeding;
· postpartum hemorrhages.
“Unlike hemophilia, deep subcutaneous hematomas, hemarthroses and intracranial hemorrhages are exceptional,” add the activists.
“Hemorrhagic manifestations appear all the earlier in life as the deficiency is deep. They nevertheless vary greatly in their intensity from one subject to another, not only depending on the type but also in individuals of the same age. family”, they continue.
The risk also varies according to the periods of life, the bleeding tendency diminishes with age in the most moderate forms (type 1) but seems to increase in type 2 subjects due to the increase in digestive bleeding.
Willebrand disease: incidence, diagnosis and treatments
In its moderate form, Willebrand disease is considered the most common familial hemorrhagic disorder, its prevalence being estimated between 0.5 and 1.1%. “However, the prevalence of symptomatic forms which require specific treatment would rather be of the order of 1 in 10,000 and as such, Willebrand disease should be considered a rare pathology”specifies the MHEMO on its website.
The diagnosis of Willebrand disease is based on the results of a set of biological tests and the existence of a personal and/or family history of bleeding. “It must be carried out by a specialized center with the various tests available and able to provide therapeutic advice adapted to the patient. adds the non-profit organization.
“The objective of the treatments is to temporarily correct the quantitative or functional deficit of the VWF”, she concludes.