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Polycythemia Vera

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NORD is very grateful to Ayalew Tefferi, MD, Professor of Medicine and Hematology, Mayo Clinic, for assistance in the preparation of this report.

Synonyms of Polycythemia Vera
  • Erythremia
  • Osler-Vaquez Disease
  • Polycythemia Rubra Vera
  • Primary Polycythemia
  • Splenomegalic Polycythemia
  • Vaquez-Osler Disease

Disorder Subdivisions



General Discussion
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.

Polycythemia vera belongs to a group of diseases known as the myeloproliferative disorders (MPDs). 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 MPDs: chronic myeloid leukemia, essential thrombocythemia and idiopathic myelofibrosis. Because the MPDs are characterized by uncontrolled cell growth, they may also be classified as blood cancers.

Organizations related to Polycythemia Vera
  • American Cancer Society, Inc.
    1599 Clifton Road NE
    Atlanta GA 30329
    Phone #: 404-320-3333
    800 #: 800-227-2345
    e-mail: N/A
    Home page: http://www.cancer.org
  • CMPD Education Foundation
    233 S. Wacker Drive, Suite 375
    Chicago IL 60606
    Phone #: 312-683-7243
    800 #: N/A
    e-mail: ian.sweet@homemail.com.au
    Home page: http://www.mpdinfo.org
  • Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg MD 20898-8126
    Phone #: 301-251-4925
    800 #: 888-205-2311
    e-mail: ordr@od.nih.gov
    Home page: http://rarediseases.info.nih.gov/Default.aspx
  • Leukemia & Lymphoma Society
    1311 Mamaroneck Avenue
    Suite 310
    White Plains NY 10605
    Phone #: 914-949-5213
    800 #: 800-955-4572
    e-mail: infocenter@LLS.org
    Home page: http://www.LLS.org
  • MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
    150 Custer Court
    Green Bay WI 54301-1243
    Phone #: 920-336-5333
    800 #: 877-336-5333
    e-mail: mums@netnet.net
    Home page: http://www.netnet.net/mums/
  • Madisons Foundation
    PO Box 241956
    Los Angeles CA 90024
    Phone #: 310-264-0826
    800 #: N/A
    e-mail: getinfo@madisonsfoundation.org
    Home page: http://www.madisonsfoundation.org
  • Myeloproliferative Disease Support and Free Daily Email Digest
    2011 Flagler Ave.
    Key West FL 33040
    Phone #: 305-295-4444
    800 #: N/A
    e-mail: roberttollen@gmail.com or mensabrain@aol.com
    Home page: http://www.MPDSUPPORT.ORG
  • Myeloproliferative Disorder (MPD) Foundation
    233 S. Wacker Drive
    Suite 375
    Chicago IL 60606
    Phone #: 312-683-7243
    800 #: N/A
    e-mail: rrosen@mpdfoundation.org
    Home page: http://www.mpdfoundation.org
  • Myeloproliferative Mailing List (MPD-SUPPORT-L)
    3706 North Roosevelt Blvd.
    Key West FL 44030
    Phone #: 305-295-4444
    800 #: --
    e-mail: lov2laf@bellsouth.net
    Home page: http://www.mpdsupport.org
  • NIH/National Heart, Lung and Blood Institute
    31 Center Drive MSC 2480
    Building 31A Rm 4A16
    Bethesda MD 20892-2480
    Phone #: 301-592-8573
    800 #: --
    e-mail: nhlbiinfo@rover.nhlbi.nih.gov
    Home page: http://www.nhlbi.nih.gov/
  • National Cancer Institute
    6116 Executive Blvd, MSC 8322, Room 3036A
    Bethesda MD 20892-8322
    Phone #: 301-435-3848
    800 #: 800-422-6237
    e-mail: N/A
    Home page: http://www.cancer.gov
  • Rare Cancer Alliance
    1649 North Pacana Way
    Green Valley AZ 85614
    Phone #: 520-625-5495
    800 #: --
    e-mail: sharon.lane@rare-cancer.org
    Home page: http://www.rare-cancer.org

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Last modified Wednesday, November 26, 2008