Clinical and laboratory diagnosis of von Willebrand disease: A synopsis of the 2008 NHLBI/NIH guidelines

Authors

  • William L. Nichols,

    Corresponding author
    1. Special Coagulation Laboratory, Division of Hematopathology, Department of Laboratory Medicine and Pathology, College of Medicine, Mayo Clinic, Rochester, Minnesota
    2. Coagulation Clinic and Comprehensive Hemophilia Center, Division of Hematology and Internal Medicine, College of Medicine, Mayo Clinic, Rochester, Minnesota
    • Special Coagulation Laboratory and Comprehensive Hemophilia Center, Hilton 200, Mayo Clinic, 200 First St SW, Rochester, MN 55905
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  • Margaret E. Rick,

    1. Hematology Service, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland
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  • Thomas L. Ortel,

    1. Division of Hematology, Department of Medicine, Duke University Medical Center, Durham, North Carolina
    2. Clinical Coagulation Laboratory, Department of Pathology, Duke University Medical Center, Durham, North Carolina
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  • Robert R. Montgomery,

    1. Blood Research Institute, Blood Center of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin
    2. Section of Pediatric Hematology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin
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  • J. Evan Sadler,

    1. Department of Medicine, Washington University, St. Louis, Missouri
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  • Barbara P. Yawn,

    1. Department of Research, Olmsted Medical Center, Rochester, Minnesota
    2. Department of Family and Community Medicine, University of Minnesota, Minneapolis, Minnesota
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  • Andra H. James,

    1. Department of Obstetrics and Gynecology, Duke University Medical Center, Durham, North Carolina
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  • Mae B. Hultin,

    1. Department of Medicine, Stony Brook University, Stony Brook, New York
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  • Marilyn J. Manco-Johnson,

    1. Department of Pediatrics, University of Colorado Denver, Aurora, Colorado
    2. Center for Cancer and Blood Disorders, Children's Hospital of Denver, Denver, Colorado
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  • Mark Weinstein

    1. Office of Blood Research and Review, Center for Biologics Evaluation and Research, US Food and Drug Administration, Rockville, Maryland
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  • Authors disclosing potential conflicts of interest are Dr. Andra James (speaking and consulting fees, and research support from CSL Behring, and honoraria from Grifols), Dr. Marilyn Manco-Johnson (speaking and consulting fees, and research support from CSL Behring), Dr. Robert Montgomery (consultant for Baxter, CSL Behring, GTI-Diagnostics, and Astra Zeneca; CSL Behring fellowship for research support), Dr. William Nichols (CSL Behring Humate-P surgical trial study, supervision of “central laboratory” activities at the Mayo Special Coagulation Laboratory, via contract through Mayo Clinical Trials Services), Dr. Thomas Ortel (speaking fee from CSL Behring), and Dr. J. Evan Sadler (consultant and Clinical Advisory Board member for Baxter). Drs. Margaret Rick and Mark Weinstein reported no potential conflicts of interest and contributed to this manuscript in their private capacities, and no official endorsement or support by the National Institutes of Health or the Food and Drug Administration is intended or should be inferred. All other authors reported no potential conflicts of interest.

Abstract

Von Willebrand factor (VWF) mediates blood platelet adhesion and accumulation at sites of blood vessel injury, and also carries coagulation factor VIII (FVIII) that is important for generating procoagulant activity. Von Willebrand disease (VWD), the most common inherited bleeding disorder, affects males and females, and reflects deficiency or defects of VWF that may also cause decreased FVIII. It may also occur less commonly as an acquired disorder (acquired von Willebrand syndrome). This article briefly summarizes selected features of the March 2008 evidence-based clinical and laboratory diagnostic recommendations from the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel for assessment for VWD or other bleeding disorders or risks. Management of VWD is also addressed in the NHLBI guidelines, but is not summarized here. The VWD guidelines are available at the NHLBI Web site (http://www.nhlbi.nih.gov/guidelines/vwd). Am. J. Hematol. 2009. © 2009 Wiley-Liss, Inc.

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