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    Hemophilia B

    Important
    It is possible that the main title of the report Hemophilia B 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

    • Christmas disease
    • Factor IX deficiency
    • royal disease

    Disorder Subdivisions

    • None

    General Discussion

    Summary
    Hemophilia B is a rare genetic bleeding disorder in which affected individuals have insufficient levels of a blood protein called factor IX. Factor IX is a clotting factor. Clotting factors are specialized proteins needed for blood clotting, the process by which blood seals a wound to stop bleeding. Individuals with hemophilia B do not bleed faster than unaffected individuals, they bleed longer. This is because they are missing a protein involved in blood clotting and are unable to effectively stop the flow of blood from the site of a wound. This is sometimes referred to as prolonged bleeding or a bleeding episode.

    Hemophilia B is classified as mild, moderate or severe. In mild cases, bleeding symptoms may occur only after surgery, major injury or a dental procedure. In some moderate and most severe cases, bleeding symptoms may occur after a minor injury or spontaneously with no known cause.

    Hemophilia B is caused by disruptions, or changes, to the factor IX gene. The factor IX gene is located on one of two sex chromosomes - the X chromosome. Males have one X chromosome and one Y chromosome and thus one altered copy of the factor IX gene in a male is enough to cause him to have hemophilia. Females have two X chromosomes and must have two altered copies of the factor IX gene to have hemophilia. Hemophilia in females is very uncommon and therefore the disorder almost always affects males. It is possible for some females with only one altered copy of the factor IX gene to have bleeding symptoms most often seen in mild hemophilia.

    Introduction
    Hemophilia B is the second most common type of hemophilia and is estimated to occur in about 1 in 25,000 male births. It affects all races equally. Hemophilia B is also known as factor IX deficiency or Christmas disease. The disorder was first reported in the medical literature in 1952 in a patient with the name of Stephen Christmas. The most famous family with hemophilia B was that of Queen Victoria of England. Through her descendants, the disorder was passed down to the royal families of Germany, Spain and Russia and thus hemophilia B is also known as the "royal disease."

    Although the focus of this report is the genetic, or inherited, form of hemophilia B, it should be noted that another form called acquired hemophilia B can develop later in life (see "Related Disorders" section below). An individual with acquired hemophilia B is not born with the condition. Acquired hemophilia B is caused by the body's production of antibodies against its own factor IX protein. The factor IX antibodies destroy circulating factor IX in the blood causing bleeding symptoms. Acquired hemophilia B is extremely rare; most cases of acquired hemophilia are in those with hemophilia A.

    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

    Canadian Hemophilia Society
    400-1255 University Street
    Montreal
    Quebec, H3B 3B6
    Canada
    Tel: 5148480503
    Fax: 5148489661
    Tel: 8006682686
    Email: chs@hemophilia.ca
    Internet: http://www.hemophilia.ca

    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

    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/

    Madisons Foundation
    PO Box 241956
    Los Angeles, CA 90024
    Tel: (310)264-0826
    Fax: (310)264-4766
    Email: getinfo@madisonsfoundation.org
    Internet: http://www.madisonsfoundation.org

    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: 8/28/2012
    Copyright 1987, 1988, 1989, 1995, 1996, 2000, 2007, 2008, 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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