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by Scholten A

Primary Polycythemia

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

Definition

Polycythemia is a condition of the bone marrow. It makes too many red blood cells and platelets. Sometimes, white blood cells are affected. The increase of blood cells can make the blood thicken and clot.
Early treatment lowers the risk of serious problems.
Location of Active Bone Marrow in an Adult
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Causes

This condition is caused by changes to a gene. It is not clear what causes this to happen.

Risk Factors

Polycythemia is more common in men and people aged 40 years or older. The risk is higher in people of Ashkenazi Jewish decent.

Symptoms

Some people with polycythemia have no symptoms. Others may have symptoms such as:
  • Night sweats or hot flashes
  • Reddish skin color
  • Burning feeling in feet and hands
  • Weight loss
  • Bone pain or headaches
  • Dizziness, tiredness, or weakness
  • Problems with breathing, vision, or thinking
  • Ringing in the ears—tinnitus
Blood clots increase the risk of a heart attack or stroke.

Diagnosis

Polycythemia is sometimes found during a routine blood test. The doctor will ask about symptoms and past health. A physical exam will be done.
Tests may include:

Treatment

Treatment depends on how severe the disease is. The goal is to manage the disease and problems linked to it—such as blood clots. For some, a combination of treatments works best.
Options may be:
  • Medicines, such as:
    • Low-dose aspirin—to reduce the risk of blood clots
    • Chemotherapy—to lower blood cell production, control blood thickness, and improve the immune system.
    • JAK inhibitors—to reduce spleen size and improve the balance of other blood cells
  • Blood taken from the veins at times—to lower the amount of red blood cells
  • Surgery to remove the spleen—if it is causing problems

Prevention

There are no guidelines prevent polycythemia.

RESOURCES

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

CANADIAN RESOURCES

Health Canada
https://www.canada.ca
HealthLink BC
https://www.healthlinkbc.ca

References

Mesa RA. Refining the management of polycythemia vera. Clin Adv Hematol Oncol. 2018;16(9):587-589.
Polycythemia vera. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/polycythemia-vera. Accessed September 10, 2021.
Polycythemia vera. Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/hematology-and-oncology/myeloproliferative-disorders/polycythemia-vera. Accessed September 10, 2021.
Polycythemia vera. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health-topics/polycythemia-vera. Accessed September 10, 2021.

Revision Information

  • Reviewer: EBSCO Medical Review Board Nicole Meregian, PA
  • Review Date: 07/2021
  • Update Date: 09/10/2021