Female doctor talking to male patients

Von Willebrand
Disease

Von Willebrand Disease (VWD), an inherited disorder characterized by a quantitative deficiency or qualitative defect of von Willebrand Factor (VWF), is the most prevalent inherited bleeding disorder.1,2,3

VWD presents with a broad range of bleeding phenotypes, and this contributes to difficulty in diagnosis and potential delays of 15 years or more from the onset of bleeding symptoms to receiving diagnosis and treatment.3,4

Blood disorder educational diagram

Epidemiology

There are an estimated 23 to 110 cases of VWD per million people.5 Population-based estimates are higher, ranging from 0.6-1.3% of people.

There are 3 types of VWD.2,6 Type 1 VWD, which accounts for ~75% of cases, is characterized by a reduced amount of VWF and with mild or moderate bleeding episodes.

Type 2 VWD, which accounts for ~25% of cases, is characterized by qualitative defects of VWF that impair its function.2,6 This is associated with bleeding episodes that can range from mild to severe.

Type 3 VWD accounts for <5% of cases, and is characterized by a near complete quantitative deficiency of VWF in plasma and with severe bleeding episodes.2,6

Pathophysiology

Von Willebrand Factor (VWF) mediates platelet adhesion and stabilizes FVIII.1,7

In primary hemostasis, VWF mediates platelet adhesion to damaged vascular subendothelium by binding to exposed collagen and platelet receptors such as GPIbα and αIIbβ.1,7

In secondary hemostasis, VWF stabilizes FVIII in circulation by the formation of non-covalently bound VWF:FVIII complexes, thus preventing the rapid clearance of FVIII and increasing the half-life of FVIII.1,7

The quantitative or qualitative defects in VWF that are seen in VWD can lead to an inability to form platelet plugs (primary hemostasis) and to protect FVIII from proteolytic degradation (secondary reduction in FVIII levels), therefore leading to symptoms of bleeding.1,8

Diagnosis

The 2021 guideline recommendations from the American Society of Hematology (ASH), International Society on Thrombosis and Haemostasis (ISTH), National Hemophilia Foundation (NHF), and World Federation of Hemophilia (WFH) has outlined the criteria for confirmatory diagnosis and differential diagnosis of VWD subtypes.4

For individuals with low probability of VWD, such as those seen in primary care, a validated bleeding assessment tool (BAT) is recommended as an initial screening to see who needs specific blood tests.4 For those with intermediate risk (those who have been referred to a hematologist because of bleeding history) and for those with high risk (those with an affected first-degree relative), specific blood testing for VWD is recommended:

  • VWF:Ag
  • platelet-dependent VWF activity (e.g., VWF:GPIbM)
  • FVIII:C

Specific blood tests, followed by additional testing if necessary, can rule out VWD or confirm the diagnosis and subtype of VWD.4

Navigating VWD

VWD can manifest in a diverse range of bleeding symptoms:5,8,9,10

  • Heavy menstrual bleeding, defined as abnormal menstrual blood loss of >80 mL and characterized by:
    • Blood clots >1 inch in diameter
    • Changing a sanitary pad or tampon more frequently than hourly
    • Anemia
  • Spontaneous gastrointestinal bleeding requiring medical attention, or resulting in acute or chronic anemia
  • Epistaxis: ≥2 episodes without a history of trauma not stopped by short compression of <10 min, or ≥1 episode requiring blood transfusion
  • Bleeding following dental or surgical procedures such as tooth extraction, tonsillectomy and adenoidectomy
  • Joint bleeds, which may lead to structural joint damage:
    • Synovial iron deposition and inflammatory cell proliferation provoke degeneration of joint surfaces and eventual loss of normal function

Following diagnosis of VWD and its type, the guidelines outline best practices for a variety of VWD manifestations.11 This may range from prophylaxis for patients with a history of severe bleeds, on-demand treatment recommendations for other patients, and perioperative precautions for all patients.

Scientific Congresses and Resources

This is not intended to be a comprehensive resource of all congresses and congress materials across therapeutic and disease areas. Congress materials may include information about investigational use(s) of compounds/products that are not approved for use by the U.S. Food and Drug Administration (FDA) and/or are inconsistent with the Prescribing Information. Takeda does not recommend the use of any Takeda product beyond the approved labeling. Any decisions regarding the usage of a Takeda product beyond the approved labeling are left to the discretion of the healthcare professional. Takeda makes no representations about whether investigational compounds or unapproved uses will be approved by the FDA.

American Thrombosis and Hemostasis Network Virtual Data Summit (ATHN), 2025

October 21 - 23, 2025 | Link to Event

Brings together leading experts in blood disorders research and top clinicians to share their perspectives through ground-breaking presentations and robust Q&A sessions. Together, we’ll explore how ATHN’s unique data infrastructure supports research projects that leverage data to answer scientifically and clinically meaningful questions.

American Society of Hematology (ASH), 2025

December 6 - 9, 2025 | Link to Event

The premier global congress from the world's largest professional society serving both clinicians and scientists working in malignant and classical hematology.

Thrombosis and Hemostasis Societies of North America (THSNA), 2026

March 19 - 21, 2026 | Link to Event

The THSNA Summit is a collaboration of the 13 leading non-profit organizations in the fields of Thrombosis and Hemostasis. The Summit provides a focused forum for over 1,000 attendees with an interest in bleeding and clotting disorders to network, learn, and share across disciplines and disease states. The educational programming is organized in a series of plenary presentations, educational track session, oral abstract presentations and digital poster sessions.

American Society of Pediatric Hematology/Oncology (ASPHO), 2026

April 29 - May 2, 2026 | Link to Event

Conference delivers vital education and networking opportunities to subspecialist investigators and practitioners from every sector of the field as well as hospitalists, physician assistants, advanced nurse practitioners, other advanced practice providers, and allied healthcare professionals.

BIC International Conference (BIC International), 2025

September 12 - 14, 2025

The BIC International Conference is traditionally addressed to a relatively narrow selection of medical specialties since it is meant to feature only the most novel advances of basic science and clinical research in haemophilia, rare inherited coagulation disorders, von Willebrand factor and disease, gene therapy and thrombotic microangiopathies.

Adzynma (ADAMTS13, recombinant-krhn)

  • Recombinant ADAMTS13 Prophylaxis for the Treatment of Pediatric Patients with Congenital Thrombotic Thrombocytopenic Purpura: Pooled Outcomes from Two Phase 3 Studies

International Society on Thrombosis and Haemostasis (ISTH), 2025

June 21 - 25, 2025

Global congress featuring the world’s leading experts on thrombosis, hemostasis and vascular biology presenting the most recent advances, the latest science, and the newest clinical applications designed to improve patient care.

Academy of Managed Care Pharmacy (AMCP), 2025

March 30 - April 3, 2025

Annual event with more than 2,500 members and non-members of the AMCP to engage on the latest innovations and most intentional networking in managed care pharmacy.

National Comprehensive Cancer Network® (NCCN), 2025

March 28 - 30, 2025

Congress focusing on the practical management of patients living with or at risk for cancer and best practices for cancer care delivery.

Hemostasis and Thrombosis Research Society (HTRS), 2025

March 13 - 15, 2025

Scientific meeting open to all medical, research, and allied health professionals interested in disorders of hemostasis and thrombosis.

American Society of Hematology (ASH), 2024

December 7 - 10, 2024

The premier global congress from the world's largest professional society serving both clinicians and scientists working in malignant and classical hematology.

Adzynma (ADAMTS13, recombinant-krhn)

  • Pharmacodynamic Activity Of Recombinant Adamts13 Versus Plasma-Based Therapies In Congenital Thrombotic Thrombocytopenic Purpura: Interim Results Of A Phase 3 Randomized, Controlled, Open-Label Study
  • Mutation Analysis of the ADAMTS13 gene in patients with Congenital Thrombotic Thrombocytopenic Purpura from the rADAMTS13 Phase 3 study

American Thrombosis and Hemostasis Network Data Summit (ATHN Data Summit), 2024

October 22 - 23, 2024

Annual meeting discussing important research areas of hemophilia, health equity, thrombosis, Von Willebrand disease, rare blood disorders, and data management.

Videos

Watch videos focused on von Willebrand disease (VWD).

The Role of von Willebrand Factor in Hemostasis

Learn about von Willebrand's dual role in hemostasis, along with its process of storage, secretion and cleavage in this animated video.

Gastrointestinal Bleeding in von Willebrand Disease

Explore the mechanisms, clinical presentation, and management of gastrointestinal bleeding in patients with von Willebrand disease from a clinician's perspective in this animated video.

Factor VIII and von Willebrand Factor Independence

Explore the clinical relationship between Factor VIII and von Willebrand Factor from a healthcare professional’s perspective.

 

Additional Resources

Find materials to help foster a deeper understanding of von Willebrand disease (VWD).

von Willebrand Disease Milestones

A timeline of milestones from disease discovery in 1926 to current guidelines and management approaches.

Medications

This resource provides information on Takeda medications available in the Von Willebrand Disease category and is not intended to represent a complete list of therapeutic options.

Vonvendi®

[von Willebrand factor (Recombinant)]

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