Von Willebrand syndrome

Von Willebrand syndrome (VWS) is a hereditary bleeding disorder that manifests in hummhagic diatheses. There is a quantitative, structural or functional change in the Von Willebrand Factor (VWF) (named after the Finnish physician Erik Adolf von Willebrand).

Two major groups are distinguished: the quantitative disorders (partial deficiency, type 1, complete deficiency, type 3) and qualitative disorders (type 2) with several subtypes. Type 1 is autosomal dominant, while type 2 and 3 are autosomal recessive. Men and women are affected by the disease in the same rate.

Often the disease is diagnosed too late, and the expression and severity can be very different. The most common symptoms are nosebleeds, bleeding gums, hematomas and malignancies.

Women suffer from increased menstrual bleeding or bleeding complications at delivery. In severe cases, there may be bleeding in the gastrointestinal tract or kidney system and muscle bleeding.