Primary Polycythemia

(Polycythemia Vera [PCV]; Polycythemia Rubra Vera [PRV]; Erythremia)

Pronounced: pol-ee-si-thee-me-a

Definition

Polycythemia is a condition that occurs when the bone marrow produces an abnormal and excessive amount of red blood cells and platelets in the blood. The abnormal increase of red blood cells in the blood can cause the blood to thicken and clot.

There are several forms of polycythemia—primary polycythemia, secondary polycythemia, and stress polycythemia. Each form has its own set of causes and risk factors.

Location of Active Bone Marrow in an Adult

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Causes    TOP

Nearly all cases of primary polycythemia are believed to be caused by a mutation in the janus kinase 2 gene (JAK2).

Risk Factors    TOP

Primary polycythemia is more common in men and people over the age of 40 years of age. It is also more common in people who are Caucasian or those of Ashkenazi Jewish descent.

Symptoms    TOP

The symptoms of primary polycythemia occur gradually and vary from person to person. Some people show no signs of the condition. Symptoms may include:

  • Abnormal and heavy bleeding due to a cut or a nosebleed
  • Intense and frequent bone pain and/or muscle pain
  • Headaches, lightheadedness, or gastrointestinal symptoms
  • Vision problems
  • Ringing in the ears
  • Difficulty or labored breathing
  • The color of the skin appears reddish
  • Fatigue
  • Inability to concentrate
  • Intense itching after a warm or hot bath, shower, or any activity that requires soaking your skin in warm or hot water

Diagnosis    TOP

You will be asked about your symptoms and medical history. A physical exam will be done. You may be referred to a doctor called a hematologist who specializes in blood diseases and disorders. The hematologist will perform a tests.

Your bodily fluids may be tested. This can be done with:

Treatment    TOP

Talk with your doctor about the best treatment plan for you. Treatment options include the following:

Phlebotomy

Phlebotomy is a technique that requires a person to periodically have blood removed from a vein to decrease the overall total proportion of red blood cells in the body. The frequency of the treatment is based on how quickly a person’s bone marrow produces an excessive amount of red blood cells.

Chemotherapy

Chemotherapy is a group of drugs that can be given by mouth, by injection, or through a tube that is inserted into a duct or blood vessel to slow the rapid development of red blood cells in the bone marrow.

Aspirin    TOP

You may be given aspirin to reduce your risk of blood clots and spleen removal.

Prevention    TOP

There are no known preventative measures that will reduce your chances of getting primary polycythemia.

There is no cure, but recent research suggests that individuals who have primary polycythemia may significantly increase their overall life expectancy by seeking treatment right away.

RESOURCES:

American Society of Hematology
http://www.hematology.org
The National Heart, Lung, and Blood Institute
http://www.nhlbi.nih.gov

CANADIAN RESOURCES:

References:

Berk PD, Goldberg JD, et al. Therapeutic recommendations in polycythemia vera based on Polycythemia Vera Study Group protocols. Semin Hematol. 1986;23:132-143.
Gilbert HS. Current management in polycythemia vera. Semin Hematol. 2001;38(1 Suppl 2):25-28.
Golden C. Polycythemia vera: a review. Clin J Oncol Nurs. 2003;7:553-556.
Kessler CM. Propensity for hemorrhage and thrombosis in chronic myeloproliferative disorders. Semin Hematol. 2004;41(2 Suppl 3):10-14.
Munson BL. Myths & facts…about polycythemia vera. Nursing. 2005;35:28.
Passamonti F, Rumi E, et al. Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia. Am J Med. 2004;117:755-761.
Polycythemia vera. EBSCO DynaMed website. Available at:
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Updated February 23, 2015. Accessed June 30, 2015.
Ruggeri M, Tosetto A, et al. The rate of progression to polycythemia vera or essential thrombocythemia in patients with erythrocytosis or thrombocytosis. Ann Intern Med. 2003;139:470-475.
Solberg LA Jr. Therapeutic options for essential thrombocythemia and polycythemia vera. Semin Oncol. 2002;29(3 Suppl 10):10-15.
Spivak JL. The optimal management of polycythaemia vera. Br J Haematol. 2002;116:243-254.
Stuart BJ, Viera AJ. Polycythemia vera. Am Fam Physician. 2004;69:2139-2144.
Tefferi A. A contemporary approach to the diagnosis and management of polycythemia vera. Curr Hematol Rep. 2003;2:237-241.
Tefferi A. Polycythemia vera: a comprehensive review and clinical recommendations. Mayo Clin Proc. 2003;78:174-194.
Last reviewed June 2015 by Igor Puzanov, MD; Michael Woods, MD
Last Updated: 5/11/2013

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