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    Von Willebrand Disease

    Important
    It is possible that the main title of the report Von Willebrand Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

    Synonyms

    • VWD

    Disorder Subdivisions

    • von Willebrand syndrome type 1
    • von Willebrand syndrome type 2A
    • von Willebrand syndrome type 2B
    • von Willebrand syndrome type 2M
    • von Willebrand syndrome type 2N
    • von Willebrand syndrome type 3

    General Discussion

    Summary
    Von Willebrand disease (VWD) is a common inherited bleeding disorder in the general population affecting males and females equally. There are three main types of VWD (VWD type 1, VWD type 2, and VWD type 3) each with differing degrees of severity and inheritance patterns. Unlike hemophilia which is characterized by joint bleeding, VWD is typically characterized by mucocutaneous bleeding. VWD is caused by a defect or deficiency in von Willebrand Factor (VWF), a large protein made up of multiple subunits. VWF binds to clotting factor VIII in the circulation and protects it from being broken down. VWF also helps platelets bind to the inside of injured blood vessels. This leads to the formation of a stable blood clot which plugs an injured blood vessel and stops bleeding. If there is an insufficient quantity of VWF or if it is defective, an individual may have difficulty forming a blood clot. Most affected individuals have the relatively mild form of the disease, VWD type 1, and are not diagnosed until adulthood. A small percentage of these individuals may have prolonged bleeding during infancy or early childhood. Symptoms can include nosebleeds, bleeding from the gums, and easy bruising. In women with VWD, heavy menses frequently occurs. Affected individuals may bleed easily after injury, childbirth, and/or surgery. Bleeding from the stomach and intestines can occur but is less common.

    Introduction
    VWD was first described in the medical literature in 1926 by Dr. Erik von Willebrand, who differentiated the disorder from classic hemophilia. In addition to the genetic form, VWD can be acquired during life, often in relation to a separate underlying condition. This report deals specifically with the genetic forms. VWD types 1 and 3 may also be referred to as partial quantitative deficiency or total quantitative deficiency, respectively, because they are associated with low levels or near complete absence of VWF. VWD type 2 may also be referred to as qualitative VWD because VWF is present in normal or near normal levels, but doesn't function properly. In recent years, the understanding of the genetic factors and biochemistry associated with VWD has advanced considerably, especially for types 2 and 3. However, many questions and controversy still remain regarding the diagnosis and definition of VWD type 1 (see Causes section below).

    Resources

    National Hemophilia Foundation
    116 West 32nd Street, 11th Floor
    New York, NY 10001
    USA
    Tel: (212)328-3700
    Fax: (212)328-3777
    Tel: (800)424-2634
    Email: handi@hemophilia.org
    Internet: http://www.hemophilia.org

    NIH/National Heart, Lung and Blood Institute
    P.O. Box 30105
    Bethesda, MD 20892-0105
    Tel: (301)592-8573
    Fax: (301)251-1223
    Email: nhlbiinfo@rover.nhlbi.nih.gov
    Internet: http://www.nhlbi.nih.gov/

    World Federation of Hemophilia
    1425 René Lévesque Blvd. W. Suite 1010
    Montreal
    Quebec, H3G 1T7
    Canada
    Tel: 5148757944
    Fax: 5148758916
    Email: wfh@wfh.org
    Internet: http://www.wfh.org/index.asp?lang=EN

    Children's Cancer & Blood Foundation
    333 East 38th Street, Suite 830
    New York, NY 10016-2745
    Tel: (212)297-4336
    Fax: (212)297-4340
    Email: info@childrenscbf.org
    Internet: http://www.childrenscbf.org/

    Hemophilia Federation of America
    210 7th St. SE
    Suite 200B
    Washington, DC 20003
    USA
    Tel: (202)675-6984
    Fax: (202)675-6983
    Tel: (800)230-9797
    Email: info@hemophiliafed.org
    Internet: http://www.hemophiliafed.org

    Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg, MD 20898-8126
    Tel: (301)251-4925
    Fax: (301)251-4911
    Tel: (888)205-2311
    TDD: (888)205-3223
    Internet: http://rarediseases.info.nih.gov/GARD/

    Irish Haemophilia Society
    First Floor
    Cathedral Court
    New Street
    Dublin, 7
    Ireland
    Tel: 353016579900
    Fax: 353016579901
    Email: info@haemophilia.ie
    Internet: http://www.haemophilia.ie/

    For a Complete Report:

    This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

    The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

    It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

    This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

    For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

    Last Updated: 11/26/2012
    Copyright 1987, 1988, 1990, 1991, 1992, 1994, 1996, 1998, 1999, 2006, 2007, 2012 National Organization for Rare Disorders, Inc.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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