Primary polycythemia; Polycythemia rubra vera; P. Vera; Myeloproliferative disorder; Erythremia; Splenomegalic polycythemia; Vaquez's disease; Osler's disease; Polycythemia with chronic cyanosis; Erythrocytosis megalosplenica; Cryptogenic polycythemia
Polycythemia vera (PV) is a bone marrow disease that leads to an abnormal increase in the number of blood cells. The red blood cells are the most affected blood cell type.
PV is a disorder of the bone marrow. It mainly causes too many red blood cells to be produced. The numbers of white blood cells and platelets may also be higher than normal.
PV is a rare disorder that occurs more often in men than in women. It is not usually seen in people under age 40. The problem is often linked to a gene defect called JAK2V617F. The cause of this gene defect is unknown. This gene defect is not an inherited disorder.
In people with PV, there are too many red blood cells in the blood. This results in thicker blood, which can't flow through small blood vessels normally, leading to symptoms such as:
The health care provider will perform a physical exam. You may also have the following tests:
PV may also affect the results of the following tests:
PV must be distinguished from other causes of increased red cells.
The goal of treatment is to reduce the thickness of the blood and prevent bleeding and clotting problems.
A method called phlebotomy is used to decrease blood thickness. One unit of blood (about 1 pint, or 1/2 liter) is removed each week until the number of red blood cells drops. The treatment is continued as needed.
Medicines that may be used include:
Taking aspirin to reduce the risk of blood clots may be an option for some people, but aspirin increases the risk for stomach bleeding.
Ultraviolet-B light therapy can reduce the severe itching some people experience.
More information and support for people with polycythemia vera and their families can be found at:
PV usually develops slowly. Most people do not have symptoms related to the disease at the time of diagnosis. The condition is often diagnosed before severe symptoms occur.
Complications of PV may include:
Contact your provider if symptoms of PV develop.
Gotlib J. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 157.
Kremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R. The polycythemias. In: Hoffman R, Benz EJ, Silberstein LE, Heslop HE, Weitz JI, Salama ME, et al, eds. Hematology: Basic Principles and Practice. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 70.
National Cancer Institute website. Chronic myeloproliferative neoplasms treatment (PDQ) -- health professional version. www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#link/. Updated November 4, 2020. Accessed March 13, 2023.BACK TO TOP
Review Date: 2/2/2023
Reviewed By: Mark Levin, MD, Hematologist and Oncologist, Monsey, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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