Guidelines for von Willebrand Disease Urge Routine Screening for Bleeding

Abstract

FEDERAL HEALTH OFFICIALS HOPE TO raise the profile of a family of inherited bleeding disorders that are often overlooked during routine physical examinations. The National Heart, Lung, and Blood Institute (NHLBI) on February 29 issued the first US guidelines for the diagnosis and management of von Willebrand disease, the most common inherited bleeding disorder (http://www .nhlbi.nih.gov/guidelines/vwd/vwd .pdf). Patients with this disorder may experience symptoms that range from easy bruising and heavy menstrual bleeding to potentially life-threatening bleeding following surgery, trauma, or childbirth. Andra James, MD, a member of the NHLBI expert panel that wrote the guidelines, said the group hopes the document will provide physicians with a useful tool for diagnosing and managing the condition. “We wanted to raise the general awareness in the medical community that there’s a spectrum of bleeding disorders, and that even mild forms of von Willebrand disease may have implications at the time of surgery, menstruation, and childbirth,” said James, assistant professor of obstetrics and gynecology and director of the Women’s Hemostasis and Thrombosis Clinic at the Duke University School of Medicine in Durham, NC. While the guidelines, 4 years in the making, are the first to be developed for clinicians in the United States, such documents have been published for practitioners in Canada, Italy, and the United Kingdom. The disease, which the NHLBI panel estimates affects as many as 1% of the population, is caused by a deficiency or dysfunction of von Willebrand factor, a plasma glycoprotein that assists platelets to adhere at sites of vascular injury and binds and stabilizes blood clotting factor VIII. A deficiency or dysfunction of the protein can cause increased bleeding by impairing platelet adhesion or by reducing concentrations of blood clotting factor VIII.