Polycythemia Vera

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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
• Primary Polycythemia
• Splenomegalic Polycythemia
• Vaquez-Osler Disease
• Polycythemia Rubra Vera

Disorder Subdivisions

• None

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.

Resources

Leukemia & Lymphoma Society

1311 Mamaroneck Ave

3rd Floor

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



American Cancer Society, Inc.

1599 Clifton Road NE

Atlanta, GA 30329

USA

Tel: 4043203333

Tel: 8002272345

Internet: http://www.cancer.org



NIH/National Heart, Lung and Blood Institute

31 Center Drive MSC 2480

Building 31A Rm 4A16

Bethesda, MD 20892-2480

Tel: (301)592-8573

Fax: (240)629-3246

Email: nhlbiinfo@rover.nhlbi.nih.gov

Internet: http://www.nhlbi.nih.gov/



National Cancer Institute

6116 Executive Blvd, MSC 8322, Room 3036A

Bethesda, MD 20892-8322

USA

Tel: 3014353848

Tel: 8004226237

TDD: 8003328615

Internet: http://www.cancer.gov



Myeloproliferative Mailing List (MPD-SUPPORT-L)

3706 North Roosevelt Blvd.

Key West, FL 44030

USA

Tel: 3052954444

Fax: 3052953335

Email: lov2laf@bellsouth.net

Internet: http://www.mpdsupport.org



CMPD Education Foundation

PO Box 4758

Scottsdale, AZ 85261

Tel: (480)443-1975

Fax: (480)443-1154

Email: jniblack@mpdinfo.org

Internet: http://www.mpdinfo.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

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:  9/26/2008

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Last updated:	September 26, 2008