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CME: 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.

Commercial Support Disclosure: This program is supported by an educational grant from CSL Behring.

Learners may participate in this activity free of charge.

Release Date: April 27, 2021 -- Expiration Date: April 27, 2023

Faculty: Hillary Maitland, MD, MS


Faculty introduction, disclosures

vWD introductory content

  • vWF and factor VIII

·       Molecular characteristics

·       Role in hemostasis and vWD pathophysiology

  • vWD types

vWD treatment

  • Treatment approaches
  • A focus on individualizing therapy
  • Consideration of:

·       vWD type

·       Clinical indication and goals of therapy

·       Product composition

·       Contraindications

·       Thrombosis risk

  • Discussion of subtypes and their clinical differences in providing treatment
  • Surgery and prophylactic vWF replacement
  • Management of menorrhagia with vWF

Patient case(s)

Summary, conclusions, and best practice recap

Learning Objectives

By the end of the session the participant will be able to:

  • Describe the clinical management of vWD.
  • Recall patient characteristics and clinical circumstances that direct the individualizing of therapy for patients with vWD.
  • Formulate treatment strategies for patients with vWD.


ACCME Activity #201748767


Faculty Disclosure and Resolution of COI

As a provider of continuing medical education, it is the policy of ScientiaCME to ensure balance, independence, objectivity, and scientific rigor in all of its educational activities. In accordance with this policy, faculty and educational planners must disclose any significant relationships with commercial interests whose products or devices may be mentioned in faculty presentations, and any relationships with the commercial supporter of the activity. The intent of this disclosure is to provide the intended audience with information on which they can make their own judgments. Additionally, in the event a conflict of interest (COI) does exist, it is the policy of ScientiaCME to ensure that the COI is resolved in order to ensure the integrity of the CME activity. For this CME activity, any COI has been resolved thru content review ScientiaCME.

Faculty Disclosures: Hillary S. Maitland, MD, MS, Associate Professor of Medicine
University of Virginia Division of Hematology/Oncology, has received financial compensation as a consultant or as a speaker from Dova and Sanofi.

Disclosures of Educational Planners: Charles Turck, PharmD, BCPS, BCCCP, CEO of ScientiaCME, has no relevant conflicts of interest to disclose.

Commercial Support Disclosure: This program is supported by an educational grant from CSL Behring.


  • Read the learning objectives above
  • Take the Pre-Test (optional). Completion of the pre-test will help us evaluate the knowledge gained by participating in this CME activity.
  • View the online activity. You may view this is in more than one session, and may pause or repeat any portion of the presentation if you need to.
  • Minimum participation threshold: Take the post-test. A score of 70% or higher is required to pass and proceed to the activity evaluation.
  • Complete the activity evaluation and CME registration. A CE certificate will be emailed to you immediately.

Cultural/Linguistic Competence & Health Disparities

System Requirements

Windows 7 or above
Internet Explorer 8
*Adobe Acrobat Reader
Mac OS 10.2.8
Safari or Chrome or Firefox
*Adobe Acrobat Reader
Internet Explorer is not supported on the Macintosh

*Required to view Printable PDF Version

Perform Pre-Test (optional)

Please take a few minutes to participate in the optional pre-test. It will help us measure the knowledge gained by participating in this activity.

Additional Courses That Are Related To This Activity

von Willebrand's disease (vWD): therapeutic updates and optimizing treatment