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.
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.
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
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