CME: von Willebrand’s Disease (vWB): Therapeutic Updates and Optimizing Treatment
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In this online, self-learning activity:
Von Willebrand disease (vWD) is the most common congenital bleeding disorder worldwide. Affecting both male and female births in equal number, vWD is caused by a deficiency or defect in the von Willebrand factor (vWF) glycoprotein, which is responsible for mediating platelet and coagulation factor VIII function. vWD types 1 and 3 are caused by quantitative deficiencies in vWF. In contrast, type 2 vWD is caused by a qualitative defect in the production of vWF. Type 1 is the most common type of vWD, accounting for 60% to 70% of cases, followed by type 2, which is diagnosed in 25% to 30% of patients. Type 3 vWD, the rarest form, affects about 1 in 1,000,000 people. There is evidence that the use of factor VIII/vWF concentrates should be individualized, but no recent vWD guidelines address this issue. Although DDAVP is the treatment of choice for most type 1 vWD patients, data do not support the use of DDAVP for type 2B vWD owing in part to an increased risk for thrombocytopenia. Another practice gap is a lack of guidance around the appropriate ages at which patients with severe vWD are optimally initiated on vWF prophylaxis. Furthermore, although DDAVP is not contraindicated in pregnancy, 31% of physicians consider DDAVP a contraindication according to the results of one survey, illustrating a present area of controversy in practice.
The following healthcare professionals: Hematologists and primary care physicians; physician assistants, nurse practitioners, and pharmacists who practice in hematology; and any other healthcare professionals with an interest in or who clinically encounter patients with vWD.