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Polycythemia Vera

Last updated Dec. 17, 2018

Approved by: Maulik P. Purohit MD MPH

Microscopic pathology image of a blood smear from a 68-year-old woman with a 13-year history of polycythemia vera treated with phlebotomy, 32-P, and hydroxyurea.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Erythremia
  • Spenomegalic Polycythemia
  • Vaquez-Osler Disease

What is Polycythemia Vera? (Definition/Background Information)

  • Polycythemia Vera is a blood-related disorder, in which there is too much red blood cells being produced by the body
  • The bone marrow is responsible for producing red blood cells, whose function is to distribute oxygen to the body. However, with this disorder there is an increased amount of red blood cells being produced, causing the blood to thicken
  • The increased thickness of the blood decreases its fluidity, consequently reducing its flow rate, especially through the smaller blood vessels
  • A decrease or lack of blood flow, can lead to several problems, such as weakness and fatigue

Who gets Polycythemia Vera? (Age and Sex Distribution)

  • Polycythemia Vera is an uncommon blood disorder that is more prevalent in males compared to females
  • The condition is more likely to appear in individuals over the age of 60, and less likely to occur in those below 20 years of age
  • There is no evidence indicating the condition to be more common among any specific racial/ethnic group. All racial and ethnic groups are equally affected

What are the Risk Factors for Polycythemia Vera? (Predisposing Factors)

  • The risk for developing Polycythemia Vera is more in males compared to females, especially those individuals, over the age of 60 years
  • Individuals, who have a family history that demonstrates evidence of the disease among close relatives, have an increased probability of developing the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Polycythemia Vera? (Etiology)

The cause of Polycythemia Vera can vary. It can be due to a genetic defect affecting the bone marrow, or due to hormonal problems.

  • The most common cause is due to a gene mutation, which affects the bone marrow, causing it to produce more red blood cells than what is normally required for the body
  • Although research shows that genetic mutation is the most common cause for the development of the disease, the main mechanism of how it targets the bone marrow, is still unclear
  • Another cause factor is when there is an abnormality with the hormone erythropoietin, being produced by the kidney. The role of erythropoietin is to activate the bone marrow in order that it releases the red blood cells. If there is an abnormal signal from the brain to the kidney to produce excess erythropoietin, then this will result in excess amount of red blood cells in the body

What are the Signs and Symptoms of Polycythemia Vera?

Not all individuals with Polycythemia Vera have signs and symptoms. Individuals, who demonstrate signs and symptoms, have the following:

  • Trouble breathing, feeling weak and tired
  • Easy bruising and bleeding
  • Severe, throbbing headache
  • Skin appears red, or skin color changes due to lack of oxygen (blue)
  • Visual problems, difficulty speaking
  • Feeling confused, lack of balance and coordination

How is Polycythemia Vera Diagnosed?

Polycythemia Vera is diagnosed by performing appropriate history and physical examination of the individual, and using the following tests:

  • Blood works to assess the function and production of the red blood cells
  • Blood test to assess the amount of red blood cells in the body
  • Testing for blood hematocrit is used to indicate the volume of red blood cells present in the body
  • A hemoglobin test is performed to assess, if there is sufficient oxygen being distributed by the red blood cells to the body
  • The level of hormone erythropoietin is measured, since the hormone is responsible for sending signals to the bone marrow to release red blood cells
  • Studies may be performed on the bone marrow to identify whether the defect is due to bone marrow or caused by other factors, such as abnormal hormonal levels.
  • In addition to all these tests, a genetic screen test may be conducted to check for gene mutation, as this could be a cause factor in the development of the disorder

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Polycythemia Vera?

The possible complications associated with Polycythemia Vera include:

  • Formation of clots due to the accumulation of red blood cells, thereby decreasing its fluidity. This can lead to a stroke, heart attack, or cause a blockage in any of the body arteries
  • The spleen may be enlarged (splenomegaly) due to the stress placed on it. The spleen is an organ that is responsible for removing old red blood cells from circulation. However, because there is an increased amount of red blood cells in the body, the spleen is pressured to do more work causing it enlarge
  • Increase in the amount of red blood cells leads to abnormal functioning of other organs, such as stomach, small intestine, or esophagus
  • Other complications associated with Polycythemia Vera include, gout and certain types of leukemia

How is Polycythemia Vera Treated?

  • The treatment of Polycythemia Vera is focused on methods to reduce the thickness (or viscosity) of the blood and prevent possible formation of clots, which can cause more serious medical issues
  • Phlebotomy: During this procedure, blood, normally one unit, is removed at a time every week, until the blood is thinned out
  • In addition to this, individual may be put on aspirin, as it will decrease the chance of forming blood clots

How can Polycythemia Vera be Prevented?

  • Polycythemia Vera cannot be prevented but there are treatment options available to decrease the symptoms and avoid progression of the condition to a chronic form
  • Factors that may cause additional harm, or aggravate the condition, should be avoided. These include smoking, avoiding high elevated areas, or participation in sports, such as mountain climbing; since, enough oxygen may not be available at such high altitudes

What is the Prognosis of Polycythemia Vera? (Outcomes/Resolutions)

  • Polycythemia Vera prognosis depends on early diagnosis and appropriate intervention/treatment. In such cases the prognosis is better
  • In many cases, the individuals do not present many symptoms and they also respond well to treatment
  • Individuals with few/mild symptoms (associated with Polycythemia Vera), do better than those who have many or severe symptoms

Additional and Relevant Useful Information for Polycythemia Vera:

  • Polycythemia Vera is a condition that may necessitate medical monitoring for the rest of your life
  • Regular check up with you primary care physician may be necessary to maintain the appropriate red cell mass (thickness of the blood)

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Aug. 10, 2013
Last updated: Dec. 17, 2018