Polycythemia Vera

Polycythemia vera (PV), or myeloproliferative disease, is a form of blood cancer in which the bone marrow makes too many (overproduces) red blood cells. The bone marrow may also make too many clotting cells (platelets) and white blood cells. Bone marrow is the spongy center of bones where blood cells are produced. Sometimes, there may be an overproduction of blood cells in the liver and spleen, causing those organs to become enlarged. Additionally, people who have PV are at a higher risk for stroke or heart attack because their blood may clot more easily. PV is a long-term disease.

What are the causes?

Almost all people who have PV have an abnormal gene (genetic mutation) that causes changes in the way that the bone marrow makes blood cells. This gene, which is called JAK2, is not passed along from parent to child (is not hereditary). It is not known what triggers the genetic mutation that causes the body to produce too many red blood cells.

What increases the risk?

This condition is more likely to develop in:

• Males.
• People who are 60 years of age or older.

What are the signs or symptoms?

You may not have any symptoms in the early stage of PV. When symptoms develop, they may include:

• Shortness of breath.
• Dizziness.
• Hot and flushed skin.
• Itchy skin.
• Sweats, especially night sweats.
• Headache.
• Tiredness.
• Ringing in the ears.
• Blurred vision or blind spots.
• Bone pain.
• Weight loss.
• Fever.
• Blood-tinged vomit or bowel movements.

How is this diagnosed?

This condition may be diagnosed during a routine physical exam if you have a blood test called a complete blood count (CBC). Your health care provider also may suspect PV if you have symptoms. During the physical exam, your provider may find that you have an enlarged liver or spleen. You may also have tests to confirm the diagnosis. These may include:

• A procedure to remove a sample of bone marrow for testing (bone marrow biopsy).
• Blood tests to check for:
  • The JAK2 gene.
  • Low levels of a hormone that helps to regulate blood production (erythropoietin).

How is this treated?

There is no cure for PV, but treatment can help to control the disease. There are several types of treatment. No single treatment works for everyone. You will need to work with a blood cancer specialist (hematologist) to find the treatment that is best for you. Options include:

• Periodically having some blood removed with a needle (drawn) to lower the number of red blood cells (phlebotomy).
• Medicine. Your health care provider may recommend:
  • Low-dose aspirin to lower your risk for blood clots.
  • A medicine to reduce red blood cell production (hydroxyurea).
  • A medicine to lower the number of red blood cells (interferon).
  • A medicine that slows down the effects of JAK2 (ruxolitinib).

Follow these instructions at home:

• Take over-the-counter and prescription medicines only as told by your health care provider.
• Return to your normal activities as told by your health care provider. Ask your health care provider what activities are safe for you.
• Do not use tobacco products, including cigarettes, chewing tobacco, or e-cigarettes. If you need help quitting, ask your health care provider.
• Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if:

• You have side effects from your medicines.
• Your symptoms change or get worse at home.
• You have blood in your stool or you vomit blood.

Get help right away if:

• You have sudden and severe pain in your abdomen.
• You have chest pain or difficulty breathing.
• You have signs of stroke, such as:
  • Sudden numbness.
  • Weakness of your face or arm.
  • Confusion.
  • Difficulty speaking or understanding speech.

These symptoms may represent a serious problem that is an emergency. Do not wait to see if the symptoms will go away. Get medical help right away. Call your local emergency services (911 in the U.S.). Do not drive yourself to the hospital.