New Guidelines Issued for Von Willebrand Disease

Management of the bleeding disorder depends on disease subtype and severity of bleeding

MONDAY, March 10 (HealthDay News) -- New guidelines from the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel, published in the March issue of Haemophilia, call for an individualized approach to the diagnosis and treatment of the bleeding disorder von Willebrand disease.

The NHLBI Expert Panel on von Willebrand disease, chaired by William L. Nichols, M.D., of the Mayo Clinic in Rochester, Minn., summarized the pathophysiology and classification of von Willebrand disease and present consensus recommendations for diagnosis and management based on a comprehensive literature review and expert opinion.

In von Willebrand disease, decreased or dysfunctional von Willebrand factor, a protein involved in blood clotting, leads to prolonged mucosal and skin wound bleeding. In addition, there are several subtypes of von Willebrand disease, which vary in pathophysiology and severity. Diagnosis should be guided by a careful clinical history including a family history of bleeding, physical exam findings and laboratory evaluation. Management of von Willebrand disease, which is directed at controlling bleeding or providing prophylaxis prior to surgical procedures, is dependent upon subtype of von Willebrand disease as well as disease severity.

"Treatment of von Willebrand disease in the United States varies widely and frequently is based on local experience and physician preference. Few standard recommendations exist to guide therapy for von Willebrand disease," write the authors. "This guideline document presents recommendations regarding the management and prevention of bleeding in persons with von Willebrand disease and reviews the strength of evidence supporting those recommendations."

Several study co-authors report receiving consulting fees and research support from CSL Behring and other pharmaceutical companies.

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