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

von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients. These evidence-based guidelines from the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National...

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Published inBlood advances Vol. 5; no. 1; pp. 301 - 325
Main Authors Connell, Nathan T., Flood, Veronica H., Brignardello-Petersen, Romina, Abdul-Kadir, Rezan, Arapshian, Alice, Couper, Susie, Grow, Jean M., Kouides, Peter, Laffan, Michael, Lavin, Michelle, Leebeek, Frank W.G., O'Brien, Sarah H., Ozelo, Margareth C., Tosetto, Alberto, Weyand, Angela C., James, Paula D., Kalot, Mohamad A., Husainat, Nedaa, Mustafa, Reem A.
Format Journal Article
LanguageEnglish
Published United States Elsevier Inc 12.01.2021
American Society of Hematology
Subjects
Clinical Guidelines
Online AccessGet full text
ISSN2473-9529
2473-9537
2473-9537
DOI10.1182/bloodadvances.2020003264

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Abstract von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients. 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. 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. The panel agreed on 12 recommendations and outlined future research priorities. 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.
AbstractList von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients. 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. 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. The panel agreed on 12 recommendations and outlined future research priorities. 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.
von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients.BACKGROUNDvon Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients.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.OBJECTIVEThese 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.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.METHODSASH, 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.The panel agreed on 12 recommendations and outlined future research priorities.RESULTSThe panel agreed on 12 recommendations and outlined future research priorities.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.CONCLUSIONSThese 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.
Author Flood, Veronica H.
Tosetto, Alberto
Weyand, Angela C.
Kalot, Mohamad A.
Mustafa, Reem A.
Laffan, Michael
Grow, Jean M.
Brignardello-Petersen, Romina
Couper, Susie
Abdul-Kadir, Rezan
O'Brien, Sarah H.
James, Paula D.
Leebeek, Frank W.G.
Arapshian, Alice
Connell, Nathan T.
Lavin, Michelle
Kouides, Peter
Husainat, Nedaa
Ozelo, Margareth C.
Author_xml – sequence: 1
  givenname: Nathan T.
  orcidid: 0000-0003-4100-7826
  surname: Connell
  fullname: Connell, Nathan T.
  email: ntconnell@bwh.harvard.edu
  organization: Hematology Division, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA
– sequence: 2
  givenname: Veronica H.
  orcidid: 0000-0001-8998-6838
  surname: Flood
  fullname: Flood, Veronica H.
  organization: Versiti Blood Research Institute, Medical College of Wisconsin, Milwaukee, WI
– sequence: 3
  givenname: Romina
  orcidid: 0000-0002-6010-9900
  surname: Brignardello-Petersen
  fullname: Brignardello-Petersen, Romina
  organization: Department of Health Research Methods, Evidence, and Impact, McMaster University, Hamilton, ON, Canada
– sequence: 4
  givenname: Rezan
  orcidid: 0000-0002-2684-1006
  surname: Abdul-Kadir
  fullname: Abdul-Kadir, Rezan
  organization: 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
– sequence: 5
  givenname: Alice
  surname: Arapshian
  fullname: Arapshian, Alice
  organization: Middle Village, NY
– sequence: 6
  givenname: Susie
  surname: Couper
  fullname: Couper, Susie
  organization: Maylands, WA, Australia
– sequence: 7
  givenname: Jean M.
  surname: Grow
  fullname: Grow, Jean M.
  organization: Department of Strategic Communication, Marquette University, Milwaukee, WI
– sequence: 8
  givenname: Peter
  orcidid: 0000-0002-3857-8313
  surname: Kouides
  fullname: Kouides, Peter
  organization: Mary M. Gooley Hemophilia Treatment Center, University of Rochester, Rochester, NY
– sequence: 9
  givenname: Michael
  orcidid: 0000-0002-8268-3268
  surname: Laffan
  fullname: Laffan, Michael
  organization: Centre for Haematology, Imperial College London, London, United Kingdom
– sequence: 10
  givenname: Michelle
  orcidid: 0000-0003-2999-4216
  surname: Lavin
  fullname: Lavin, Michelle
  organization: Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland and National Coagulation Centre, St James's Hospital, Dublin, Ireland
– sequence: 11
  givenname: Frank W.G.
  orcidid: 0000-0001-5677-1371
  surname: Leebeek
  fullname: Leebeek, Frank W.G.
  organization: Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands
– sequence: 12
  givenname: Sarah H.
  orcidid: 0000-0001-8855-9746
  surname: O'Brien
  fullname: O'Brien, Sarah H.
  organization: Division of Hematology/Oncology, Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH
– sequence: 13
  givenname: Margareth C.
  orcidid: 0000-0001-5938-0675
  surname: Ozelo
  fullname: Ozelo, Margareth C.
  organization: Hemocentro UNICAMP, University of Campinas, Campinas, Brazil
– sequence: 14
  givenname: Alberto
  orcidid: 0000-0002-0119-5204
  surname: Tosetto
  fullname: Tosetto, Alberto
  organization: Hemophilia and Thrombosis Center, Hematology Department, S. Bortolo Hospital, Vicenza, Italy
– sequence: 15
  givenname: Angela C.
  orcidid: 0000-0003-2595-8541
  surname: Weyand
  fullname: Weyand, Angela C.
  organization: Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI
– sequence: 16
  givenname: Paula D.
  orcidid: 0000-0003-4649-9014
  surname: James
  fullname: James, Paula D.
  organization: Department of Medicine, Queen's University, Kingston, ON, Canada; and
– sequence: 17
  givenname: Mohamad A.
  orcidid: 0000-0002-6581-4561
  surname: Kalot
  fullname: Kalot, Mohamad A.
  organization: Outcomes and Implementation Research Unit, Division of Nephrology and Hypertension, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS
– sequence: 18
  givenname: Nedaa
  orcidid: 0000-0002-9638-2992
  surname: Husainat
  fullname: Husainat, Nedaa
  organization: Outcomes and Implementation Research Unit, Division of Nephrology and Hypertension, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS
– sequence: 19
  givenname: Reem A.
  orcidid: 0000-0002-2091-0875
  surname: Mustafa
  fullname: Mustafa, Reem A.
  organization: Outcomes and Implementation Research Unit, Division of Nephrology and Hypertension, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS
BackLink https://www.ncbi.nlm.nih.gov/pubmed/33570647$$D View this record in MEDLINE/PubMed
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– volume: 17
  start-page: 391
  issue: 5
  year: 2019
  ident: 2021060114533632400_B34
  article-title: A phase III study comparing secondary long-term prophylaxis versus on-demand treatment with vWF/FVIII concentrates in severe inherited von Willebrand disease
  publication-title: Blood Transfus
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Snippet von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients. These...
von Willebrand disease (VWD) is a common inherited bleeding disorder. Significant variability exists in management options offered to patients.BACKGROUNDvon...
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SubjectTerms Clinical Guidelines
Title ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease
URI https://www.clinicalkey.com/#!/content/1-s2.0-S2473952921000288
https://dx.doi.org/10.1182/bloodadvances.2020003264
https://www.ncbi.nlm.nih.gov/pubmed/33570647
https://www.proquest.com/docview/2488556182
https://pubmed.ncbi.nlm.nih.gov/PMC7805326
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