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    Polycythemia Vera

    Important
    It is possible that the main title of the report Polycythemia Vera 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

    • erythremia
    • Osler-Vaquez disease
    • polycythemia rubra vera
    • primary polycythemia
    • splenomegalic polycythemia
    • Vaquez-Osler disease

    Disorder Subdivisions

    • None

    General Discussion

    Summary
    Polycythemia vera is a rare, chronic disorder involving the overproduction of blood cells in the bone marrow (myeloproliferation). The overproduction of red blood cells is most dramatic, but the production of white blood cells and platelets are also elevated in most cases. Since red blood cells are overproduced in the marrow, this leads to abnormally high numbers of circulating red blood cells (red blood mass) within the blood. Consequently, the blood thickens and increases in volume, a condition called hyperviscosity. Thickened blood may not flow through smaller blood vessels properly. A variety of symptoms can occur in individuals with polycythemia vera including nonspecific symptoms such as headaches, fatigue, weakness, dizziness or itchy skin; an enlarged spleen (splenomegaly); a variety of gastrointestinal issues; and the risk of blood clot formation, which may prevent blood flow to vital organs. More than 90 percent of individuals with polycythemia vera have a mutation of the JAK2 gene. The exact role that this mutation plays in the development of polycythemia vera is not yet known.

    Introduction
    Polycythemia vera was first reported in the medical literature in 1892. The term "myeloproliferative disorder" (MPD) was first used to described polycythemia vera and related disorders in 1951. In 2008, the World Health Organization reclassified MPDs to "myeloproliferative neoplasms" (MPNs) to reflect the consensus that these diseases are blood cancers (neoplasms).

    This group of disorders is characterized by the overproduction (proliferation) of one or more of the three main blood cell lines - red or white blood cells or platelets. Red blood cells carry oxygen to the body. White blood cells fight infection. Platelets are involved in clotting of the blood in response to injury. Three other disorders are commonly classified as MPNs: chronic myeloid leukemia, essential thrombocythemia and idiopathic myelofibrosis. Because MPNs are characterized by uncontrolled cell growth, they may also be classified as blood cancers.

    Resources

    Leukemia & Lymphoma Society
    1311 Mamaroneck Avenue
    Suite 310
    White Plains, NY 10605
    Tel: (914)949-5213
    Fax: (914)949-6691
    Tel: (800)955-4572
    Email: infocenter@LLS.org
    Internet: http://www.LLS.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/

    National Cancer Institute
    6116 Executive Blvd Suite 300
    Bethesda, MD 20892-8322
    USA
    Tel: (301)435-3848
    Tel: (800)422-6237
    TDD: (800)332-8615
    Email: cancergovstaff@mail.nih.gov
    Internet: http://www.cancer.gov

    Rare Cancer Alliance
    1649 North Pacana Way
    Green Valley, AZ 85614
    USA
    Internet: http://www.rare-cancer.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

    Myeloproliferative Disease Support and Daily Email Digest
    2011 Flagler Ave.
    Key West, FL 33040
    USA
    Tel: (305)295-4444
    Email: roberttollen@gmail.com
    Internet: http://www.mpdsupport.org/

    MPN Research Foundation
    180 N. Michigan Avenue, Suite 1870
    Chicago, IL 60601
    Tel: (312)683-7249
    Fax: (312)332-0840
    Email: mwoerhle@MPNResearchFoundation.org
    Internet: http://www.mpnresearchfoundation.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: 5/8/2013
    Copyright 1986, 1990, 1994, 1995, 1997, 1998, 2005, 2008, 2011, 2013 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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