von Willebrand Disease

Discovering and Delivering Compassionate, Coordinated Care

The most common inherited bleeding disorder is von Willebrand disease. It is genetically transmitted from generation to generation, and is caused by a defect or deficiency in an essential blood clotting protein called the von Willebrand factor. 

While hemophilia occurs mostly in males, von Willebrand disease affects both males and females. It is estimated that between one and three percent of the U.S. population has von Willebrand disease. 

Normally, proteins called clotting factors in blood act as a patch when a blood vessel is injured. von Willebrand factor is what allows the platelets to stick to the blood vessel wall. When this factor is deficient, bleeding lasts longer. 

Easy and excessive bruising, nosebleeds, heavy or prolonged menstrual bleeding, or prolonged or excessive bleeding following a tooth extraction are the most common symptoms of von Willebrand disease. Unlike hemophilia, von Willebrand disease is more likely to cause bleeding under skin and manifests itself as severe bruising or nosebleeds. Most often it is a mild disorder. 

While von Willebrand disease is considered to be hereditary (the gene is passed from one generation to another), it can also occur in someone who has no family history of the disorder. Testing for von Willebrand disease is done at the UMass Memorial New England Hemophilia Center. Link to bleeding and clotting page 

There is no cure for von Willebrand disease but it can be treated using birth control pills, desmopressin acetate and clotting factor concentrates that can be injected or taken as a nasal spray. Minor bleeding problems, such as bruising, may not require treatment.