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Haemophilia. 2019 Nov 26. doi: 10.1111/hae.13881. [Epub ahead of print]

An international survey to inform priorities for new guidelines on von Willebrand disease.

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Outcomes and Implementation Research Unit, Department of Nephrology and Hypertension, University of Kansas Medical Center, Kansas City, KS, USA.
Hematology Division, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.
Division of Hematology/Oncology, Department of Pediatrics, Medical College of Wisconsin, Wauwatosa, WI, USA.
Department of Health Research Methods, Evidence, and Impact, McMaster University, Hamilton, ON, Canada.
Queen's University, Kingston, ON, Canada.



von Willebrand disease (VWD) is an inherited bleeding disorder caused by a quantitative or qualitative dysfunction of von Willebrand factor. Clinicians, patients and other stakeholders have many questions about the diagnosis and management of the disease.


To identify topics of highest importance to stakeholders that could be addressed by guidelines to be developed by the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF) and the World Federation of Hemophilia (WFH).


A survey to determine and prioritize topics to be addressed in the collaborative development of guidelines for VWD was distributed to international stakeholders including patients, caregivers and healthcare providers (HCPs). Representatives of the four organizations coordinated the distribution strategy. The survey focused on both diagnosis and management of VWD, soliciting 7-point Likert-scale responses and open-ended comments, in English, French and Spanish. We conducted descriptive analysis with comparison of results by stakeholder type, gender and countries' income classification for the rating questions and qualitative conventional content data analysis for the open-ended responses.


A total of 601 participants responded to the survey (49% patients/caregivers and 51% healthcare providers). The highest priority topics identified were diagnostic criteria/classification, bleeding assessment tools and treatment options for women and surgical patients. In contrast, screening for anaemia and differentiating plasma-derived therapy versus recombinant therapies received lower ratings.


This survey highlighted areas of importance to a diverse representation of stakeholders in the diagnosis and management of VWD, providing a framework for future guideline development and implementation.


bleeding disorders; guidelines; multidisciplinary; patient engagement; stakeholders; survey; von Willebrand disease


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