Nathan T Connell1, Veronica H Flood2, Romina Brignardello-Petersen3, Rezan Abdul-Kadir4, Alice Arapshian5, Susie Couper6, Jean M Grow7, Peter Kouides8, Michael Laffan9, Michelle Lavin10, Frank W G Leebeek11, Sarah H O'Brien12, Margareth C Ozelo13, Alberto Tosetto14, Angela C Weyand15, Paula D James16, Mohamad A Kalot17, Nedaa Husainat17, Reem A Mustafa17. 1. Hematology Division, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA. 2. Versiti Blood Research Institute, Medical College of Wisconsin, Milwaukee, WI. 3. Department of Health Research Methods, Evidence, and Impact, McMaster University, Hamilton, ON, Canada. 4. Department of Obstetrics and Gynaecology and Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free Foundation Hospital and Institute for Women's Health, University College London, London, United Kingdom. 5. Middle Village, NY. 6. Maylands, WA, Australia. 7. Department of Strategic Communication, Marquette University, Milwaukee, WI. 8. Mary M. Gooley Hemophilia Treatment Center, University of Rochester, Rochester, NY. 9. Centre for Haematology, Imperial College London, London, United Kingdom. 10. Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland and National Coagulation Centre, St James's Hospital, Dublin, Ireland. 11. Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands. 12. Division of Hematology/Oncology, Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH. 13. Hemocentro UNICAMP, University of Campinas, Campinas, Brazil. 14. Hemophilia and Thrombosis Center, Hematology Department, S. Bortolo Hospital, Vicenza, Italy. 15. Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI. 16. Department of Medicine, Queen's University, Kingston, ON, Canada; and. 17. Outcomes and Implementation Research Unit, Division of Nephrology and Hypertension, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS.
Abstract
BACKGROUND: von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients. OBJECTIVE: These evidence-based guidelines from 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 health care professionals in their decisions about management of VWD. METHODS: ASH, ISTH, NHF, and WFH formed a multidisciplinary guideline panel. Three patient representatives were included. The panel was balanced to minimize potential bias from conflicts of interest. The University of Kansas Outcomes and Implementation Research Unit and the McMaster Grading of Recommendations Assessment, Development and Evaluation (GRADE) Centre supported the guideline development process, including performing and updating systematic evidence reviews (through November 2019). The panel prioritized clinical questions and outcomes according to their importance to clinicians and patients. The panel used the GRADE approach, including GRADE Evidence-to-Decision frameworks, to assess evidence and make recommendations, which were subject to public comment. RESULTS: The panel agreed on 12 recommendations and outlined future research priorities. CONCLUSIONS: These guidelines make key recommendations regarding prophylaxis for frequent recurrent bleeding, desmopressin trials to determine therapy, use of antiplatelet agents and anticoagulant therapy, target VWF and factor VIII activity levels for major surgery, strategies to reduce bleeding during minor surgery or invasive procedures, management options for heavy menstrual bleeding, management of VWD in the context of neuraxial anesthesia during labor and delivery, and management in the postpartum setting.
BACKGROUND:von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients. OBJECTIVE: These evidence-based guidelines from 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 health care professionals in their decisions about management of VWD. METHODS: ASH, ISTH, NHF, and WFH formed a multidisciplinary guideline panel. Three patient representatives were included. The panel was balanced to minimize potential bias from conflicts of interest. The University of Kansas Outcomes and Implementation Research Unit and the McMaster Grading of Recommendations Assessment, Development and Evaluation (GRADE) Centre supported the guideline development process, including performing and updating systematic evidence reviews (through November 2019). The panel prioritized clinical questions and outcomes according to their importance to clinicians and patients. The panel used the GRADE approach, including GRADE Evidence-to-Decision frameworks, to assess evidence and make recommendations, which were subject to public comment. RESULTS: The panel agreed on 12 recommendations and outlined future research priorities. CONCLUSIONS: These guidelines make key recommendations regarding prophylaxis for frequent recurrent bleeding, desmopressin trials to determine therapy, use of antiplatelet agents and anticoagulant therapy, target VWF and factor VIII activity levels for major surgery, strategies to reduce bleeding during minor surgery or invasive procedures, management options for heavy menstrual bleeding, management of VWD in the context of neuraxial anesthesia during labor and delivery, and management in the postpartum setting.
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