von Willebrand’s disease: an in-depth review of management strategies
Activity Description / Statement of Need:
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.
Recent advances in replacement therapy with factor VIII/vWF concentrates have expanded treatment options. A few considerations may impact determination of best choice of agent, including hemostatic capacity and relative proportion of factor VIII. For these reasons and others, there has been a recent focus in individualizing replacement therapy in a manner that takes into account vWD type, thrombosis risk, and clinical indication and goals of therapy. Taking this information into account as well the fact that HCPs are oftentimes unable to keep up with the steady publishing of literature and evolution of clinical practice, continuing HCP education examining clinical decision-making and the appropriate selection of therapy is warranted.
Target Audience:
The following HCPs: 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.
Cost: Free
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Activity Description / Statement of Need:
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.
Recent advances in replacement therapy with factor VIII/vWF concentrates have expanded treatment options. A few considerations may impact determination of best choice of agent, including hemostatic capacity and relative proportion of factor VIII. For these reasons and others, there has been a recent focus in individualizing replacement therapy in a manner that takes into account vWD type, thrombosis risk, and clinical indication and goals of therapy. Taking this information into account as well the fact that HCPs are oftentimes unable to keep up with the steady publishing of literature and evolution of clinical practice, continuing HCP education examining clinical decision-making and the appropriate selection of therapy is warranted.
Target Audience:
The following HCPs: 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.