von Willebrand Disease

von Willebrand Disease is a common inherited bleeding disorder that affects the blood's ability to clot properly. It involves a problem with a protein called von Willebrand factor, which helps platelets stick together and form clots to stop bleeding. This condition mainly affects the blood and circulatory system. People with this disease often experience frequent nosebleeds, easy bruising, and prolonged bleeding from cuts or dental work. Women may have heavy menstrual bleeding. The severity of symptoms can vary widely, from mild to more serious bleeding episodes.

von Willebrand Disease is a hereditary bleeding disorder caused by quantitative or qualitative defects in von Willebrand factor (vWF), a glycoprotein essential for platelet adhesion and stabilization of factor VIII in the coagulation cascade. It is the most common inherited bleeding disorder and is usually inherited in an autosomal dominant pattern, although some types are recessive. The deficiency or dysfunction of vWF leads to impaired platelet adhesion to subendothelial collagen and reduced factor VIII levels, resulting in mucocutaneous bleeding and prolonged bleeding times. Clinical manifestations include epistaxis, menorrhagia, easy bruising, and bleeding after surgery or trauma. Laboratory findings typically show a prolonged bleeding time, normal or mildly prolonged PT, and variable aPTT depending on factor VIII levels. The disease is classified into types 1, 2 (with subtypes), and 3 based on the nature of the vWF defect.