Learning more about von Willebrand disease

Last updated: 12th July, 2022

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1. Overview of von Willebrand disease ­

The von Willebrand disease (vWD) is associated with disorders of hemostasis due to the fact that blood cannot clot properly. It affects around 1% of the population and occurs equally in men and women.

This disease can either occur because of a low level of the protein (von Willebrand factor) or because of altered protein activity. Both conditions prevent the von Willebrand factor (vWF) properly interacting with platelets and/or factor VIII, leading to an abnormal clotting process.

The most common symptom is abnormal bleeding, that can occur in several contexts: frequent nosebleeds; excessive bleeding following injury, dental work or surgery; presence of blood in urine; heavy menstrual bleeding.

­Depending on the type of disease, symptoms can vary a lot between patients (from mild to more severe forms). It is estimated that 1/10,000 individuals affected by vWD are symptomatic. vWD is usually an inherited genetic disorder (autosomal dominant inheritance) but can also exceptionally be de novo acquired.

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2. The different types of von Willebrand disease

There are three main types of vWD: type 1 is associated with mild to moderate bleeding, type 2 is associated with variable bleeding (mild to moderate). Type 3 is very rare but is associated with the most severe symptoms.

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3. Contribution of multimer analysis for an appropriate patient management

This disease cannot be cured. However, appropriate clinical management allows a reduction in the severity of symptoms, ensuring a quasi-normal life for affected patients. On suspicion of vWD, the clinician can prescribe several tests in order to ​confirm the disease and then to determine the type of the disease. The latter is crucial because depending on the disease type, the treatment administered to the patient can be different.

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