ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease.pdf (1.26 MB)

ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease

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posted on 30.04.2021, 13:40 by Paula D James, Nathan T Connell, Barbara Ameer, Jorge Di Paola, Jeroen Eikenboom, Nicolas Giraud, Sandra Haberichter, Vicki Jacobs-Pratt, Nicolas Konkle, Claire McLintock, Simon McRae, Robert R Montgomery, James O'Donnell, Nikole Scappe, Robert Sidonio, Veronica H Flood, Nedaa Husainat, Mohamad A Kalot, Reem A Mustafa

Background: von Willebrand disease (VWD) is the most common inherited bleeding disorder known in humans. Accurate and timely diagnosis presents numerous challenges.

Objective: These evidence-based guidelines of 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) are intended to support patients, clinicians, and other health care professionals in their decisions about VWD diagnosis.

Methods: ASH, ISTH, NHF, and WFH established a multidisciplinary guideline panel that included 4 patient representatives and was balanced to minimize potential bias from conflicts of interest. The Outcomes and Implementation Research Unit at the University of Kansas Medical Center (KUMC) supported the guideline-development process, including performing or updating systematic evidence reviews up to 8 January 2020. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE Evidence-to-Decision frameworks, to assess evidence and make recommendations, which were subsequently subject to public comment.

Results: The panel agreed on 11 recommendations.

Conclusions: Key recommendations of these guidelines include the role of bleeding-assessment tools in the assessment of patients suspected of VWD, diagnostic assays and laboratory cutoffs for type 1 and type 2 VWD, how to approach a type 1 VWD patient with normalized levels over time, and the role of genetic testing vs phenotypic assays for types 2B and 2N. Future critical research priorities are also identified.

Funding

ASH

ISTH

NHF

WFH

History

Comments

This research was originally published in Blood Advances. James PD, Connell NT, Ameer B, Di Paola J, Eikenboom J, Giraud N, Haberichter S, Jacobs-Pratt V, Konkle B, McLintock C, McRae S, Montgomery RR, O'Donnell JS, Scappe N, Sidonio R, Flood VH, Husainat N, Kalot MA, Mustafa RA. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Advances. 2021;5(1):280-300. © the American Society of Hematology.

Published Citation

James PD, Connell NT, Ameer B, Di Paola J, Eikenboom J, Giraud N, Haberichter S, Jacobs-Pratt V, Konkle B, McLintock C, McRae S, Montgomery RR, O'Donnell JS, Scappe N, Sidonio R, Flood VH, Husainat N, Kalot MA, Mustafa RA. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Advances. 2021;5(1):280-300.

Publication Date

12 January 2021

PubMed ID

33570651

Department/Unit

  • Irish Centre for Vascular Biology
  • School of Pharmacy and Biomolecular Sciences

Research Area

  • Vascular Biology

Publisher

American Society of Hematology

Version

  • Published Version (Version of Record)

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