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A.D.A.M. Medical Encyclopedia [Internet]. Atlanta (GA): A.D.A.M.; 2013.

A.D.A.M. Medical Encyclopedia.

Polycythemia vera

Primary polycythemia; Polycythemia rubra vera; Myeloproliferative disorder; Erythremia; Splenomegalic polycythemia; Vaquez's disease; Osler's disease; Polycythemia with chronic cyanosis - Myelopathic polycythemia; Erythrocytosis megalosplenica; Cryptogenic polycythemia

Last reviewed: March 3, 2013.

 Polycythemia vera is a bone marrow disease that leads to an abnormal increase in the number of blood cells. The red blood cells are mostly affected.

Causes

Polycythemia vera 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 increase as well..

This is a rare disorder that occurs more often in men than in women. It is not often 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.

Symptoms

  • Trouble breathing when lying down
  • Dizziness
  • Excess bleeding
  • Full feeling in the left upper abdomen (due to enlarged spleen)
  • Headache
  • Itchiness, especially after a warm bath
  • Red skin coloring, especially of the face
  • Shortness of breath
  • Symptoms of blood clots in veins near the skin surface (phlebitis)

Other symptoms that may occur with this disease:

  • Bluish skin color
  • Fatigue
  • Red skin spots
  • Vision problems

Exams and Tests

The health care provider will perform a physical exam. You may also have the following tests:

This disease may also affect the results of the following tests:

  • ESR
  • Lactate dehydrogenase (LDH)
  • Leukocyte alkaline phosphatase
  • Platelet aggregation test
  • Serum uric acid

Treatment

The goal of treatment is to reduce the thickness of the blood and prevent bleeding and clotting.

A method called phlebotomy is used to decrease blood thickness. One unit of blood (about 1 pint) is removed each week until the number of red blood cells drops. The treatment is continued as needed.

Other treatments may include:

Taking aspirin to reduce the risk of blood clots may be an option for some people. However, aspirin increase the risk of stomach bleeding.

Ultraviolet-B light therapy can reduce the severe itching some patients experience.

Outlook (Prognosis)

The disease usually develops slowly. Most patients do not have problems related to the disease after being diagnosed. The condition is often diagnosed before severe symptoms occur.

Possible Complications

When to Contact a Medical Professional

Call your health care provider if symptoms of polycythemia vera develop.

References

  1. Kremyanskaya M, Najfeld V, Mascarenhas J et al. The polycythemias. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Saunders Elsevier; 2012:chap 67.
  2. Tefferi A. Polycythemias. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 169.

Review Date: 3/3/2013.

Reviewed by: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.

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Copyright © 2013, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

What works?

  • Antiplatelet drugs for preventing arterial and venous thrombotic events in patients with polycythaemia vera or essential thrombocythaemiaAntiplatelet drugs for preventing arterial and venous thrombotic events in patients with polycythaemia vera or essential thrombocythaemia
    Low‐dose aspirin as an antiplatelet therapy is the drug of choice for preventing cardiovascular events, but the potential risk of bleeding has limited its use in myeloproliferative neoplasms in the past. Evidence from this review of 630 people in two trials suggests that, in patients with polycythaemia vera and with no clear indication or contraindication to aspirin therapy, low‐dose aspirin may reduce the risk of thrombotic and all‐cause mortality. No data were provided on mortality from bleeding episodes. No studies in participants with essential thrombocythaemia and with antiplatelet therapy other than aspirin have been published.
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