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Von Willebrand Disease

Overview

Von Willebrand disease (VWD) is a genetic disorder caused by missing or defective von Willebrand factor (VWF), a clotting protein. VWF binds factor VIII, a key clotting protein, and platelets in blood vessel walls, which help form a platelet plug during the clotting process. The condition is named after Finnish physician Erik von Willebrand, who first described it in the 1920s. VWD is the most common bleeding disorder. It is carried on chromosome 12 and occurs equally in men and women.1

Symptoms

People with VWD experience frequent nosebleeds, easy bruising and excessive bleeding during and after invasive procedures, such as tooth extractions and surgery. Women often experience heavy menstrual bleeding (heavy menstrual periods that last longer than average), and hemorrhaging after childbirth.1

Causes and Risk Factors

The usual cause of von Willebrand disease is an inherited abnormal gene that controls von Willebrand factor, a protein that plays a key role in blood-clotting. Rarely, von Willebrand disease can develop later in life in people who didn’t inherit an abnormal gene from a parent. This is known as acquired von Willebrand disease, and it’s likely caused by another medical condition.2

1. National Hemophilia Foundation. Von Willebrand Disease. https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Von-Willebrand-Disease
2. Mayo Clinic. Von Willebrand disease. https://www.mayoclinic.org/diseases-conditions/von-willebrand-disease/symptoms-causes/syc-20354978
3. Proposal for standardization of methods for von Willebrand factor binding to recombinant Glycoprotein Ib - an International Reference Reagent for VWF:GPIbM and VWF:GPIbR methods. https://www.who.int/biologicals/BS.2018.2337_VWFGPIb_methods.pdf
Image source: https://imagebank.hematology.org/image/60954/von-willebrand-disease-type-2b
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