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 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

Causes

A mutation in certain genes causes polycythemia.

Risk Factors

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

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

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

You may have:

A physical exam
Blood tests
Genetic tests
Bone marrow biopsy to look for overproduction of cells or other problems
Abdominal ultrasound to see if the spleen is enlarged

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

Treatment

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

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

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

Health Canada
https://www.canada.ca

HealthLink BC
https://www.healthlinkbc.ca

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.

Revision Information

Reviewer: EBSCO Medical Review Board Michael Fucci, DO, FACC
Review Date: 05/2018
Update Date: 07/17/2018

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This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.