Guidelines

The Diagnosis, Evaluation and Management of von Willebrand Disease

From the National Heart Lung and Blood Institute, National Institutes of Health

These guidelines for diagnosis and management of von Willebrand disease (VWD) were developed for practicing primary care and specialist clinicians—including family physicians, internists, obstetrician-gynecologists, pediatricians, and nurse-practitioners—as well as hematologists and laboratory medicine specialists.

Diagnosis and Evaluation

The evaluation of a person for possible VWD or other bleeding disorders may be initiated because of a variety of clinical indications. These indications and situations may include evaluation of: (1) an asymptomatic person who will undergo a surgical or interventional procedure; (2) persons who present with current symptoms of or a history of increased bleeding, abnormal laboratory studies, and/or a positive family history of a bleeding disorder; or (3) persons who present with a prior diagnosis of VWD but do not have supporting laboratory documentation. In all cases, the initial step in assessment should focus on key aspects of the person’s clinical history to determine whether the person may benefit from further diagnostic evaluation.

Management of VWD

Therapies to prevent or control bleeding in persons who have VWD follow three general strategies. The first strategy is to increase plasma concentration of VWF by releasing endogenous VWF stores through stimulation of endothelial cells with DDAVP. The second approach is to replace VWF by using human plasma-derived, viral-inactivated concentrates. The third strategy employs agents that promote hemostasis and wound healing but do not substantially alter the plasma concentration of VWF. The three treatment options are not mutually exclusive, and patients may receive any one or all three classes of agents at the same time.

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