Primary Polycythemia

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

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

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 cause the blood to thicken and clot.

There are many types. Each type has its own set of causes and risk factors. There is no exact cure. Early and proper care lowers the chances of serious problems.

Location of Active Bone Marrow in an Adult

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Causes    TOP

A mutation in certain genes causes polycythemia.

Risk Factors    TOP

Your chances of polycythemia are higher:

  • For men
  • Those aged 40 years or older
  • For people who are White
  • If you are of Ashkenazi Jewish decent

Symptoms    TOP

The symptoms of polycythemia happen slowly. They differ from person to person. Some people show no signs of the condition. Polycythemia may cause:

  • Itching after a warm or hot bath, shower, or any activity that requires soaking your skin in warm or hot water
  • Weight loss
  • Frequent bone pain or muscle pain
  • Weakness
  • Fatigue
  • Heavy bleeding from a simple cut or nosebleed
  • Headaches
  • Lightheadedness
  • Vision problems
  • Ringing in the ears—tinnitus
  • Breathing problems
  • Reddish skin color
  • Problems thinking

Blood clots increase the risk of a heart attack or stroke.

Diagnosis    TOP

The doctor will ask about your symptoms and health history. Polycythemia is sometimes found by chance during a routine blood test.

You may have:

The tests can show the severity of the disease. This helps guide a care plan.

Treatment    TOP

Care is based on severity of the disease. The goal is to control the course of the disease. This will help with managing problems caused by blood clots or bleeding. For some, a combination of methods works best.

Care may involve:

Phlebotomy

This technique allows a person to have blood removed at times. It lowers the amount of red blood cells. Phlebotomy is done when the levels get too high.

Medicines

Common medicines:

  • Chemotherapy—Lowers blood cell production, controls blood volume, and improves your immune system.
  • JAK inhibitors—Reduces spleen size and improves the balance of other blood cells.
  • Low-dose aspirin—Thins blood to reduce the risk of blood clots. Don't take aspirin without talking to your doctor. It increases the risk of gastrointestinal bleeding.

Medicines can also help ease symptoms:

  • Antihistamines or others to control itchy skin
  • Pain relievers
  • Allopurinol to reduce uric acid levels in the blood which can lead to gout

Surgery    TOP

An enlarged spleen can be removed if it’s causing problems. Common problems are pain, pressure, or higher blood pressure in the liver. Your chances of certain infections are higher without a spleen.

Prevention    TOP

There is no way to prevent polycythemia since the cause is unknown.

RESOURCES:

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

CANADIAN RESOURCES:

References:

Pasquier F, Cabagnols X, Secardin L, Plo I, Vainchenker W. Myeloproliferative neoplasms: JAK2 signaling pathway as a central target for therapy. Clin Lymphoma Myeloma Leuk. 2014;14 Suppl:S23-S35.
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 Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T113786/Polycythemia-vera. Updated March 1, 2017. Accessed July 17, 2018.
Polycythemia vera. Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/hematology-and-oncology/myeloproliferative-disorders/polycythemia-vera. Updated December 2016. Accessed July 17, 2018.
Polycythemia vera. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health-topics/polycythemia-vera. Accessed July 17, 2018.
Stuart BJ, Viera AJ. Polycythemia vera. Am Fam Physician. 2004;69(9):2139-2144.
Last reviewed June 2018 by EBSCO Medical Review Board Michael Fucci, DO, FACC
Last Updated: 7/17/2018

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