Polycythemia Vera (PV)

Polycythemia Vera (PV) is one of the myeloproliferative disorders in which the bone marrow makes too many red blood cells. It may also result in the production of too many other types of blood cells, such as white blood cells and platelets. Red blood cells carry oxygen to the body. White blood cells fight infection. Platelets are involved in clotting the blood in response to an injury. Polycythemia Vera slows your blood flow which can increase the risk of developing clots that can sometimes cause strokes.

Up to 95 percent of PV patients have the JAK2 gene mutation, which plays a significant role in the production of red blood cells, white blood cells and platelets. The discovery of this mutation has been instrumental in the development of new drugs that are currently in clinical trials. One JAK2 inhibitor (JAKAFI) was approved in 2011 for myelofibrosis and is currently being tested in PV patients. With proper care, treatments, and all around good healthy choices, many PV patients are able to live long and productive lives.

Symptoms may include:

• Headache

• Night sweats

• Weight loss

• Bone pain

• Dizziness

• Itching – especially after a warm bath or shower

• Shortness of breath

• Difficulty breathing lying down

• Numbness, tingling in hands, feet, arms and legs

• Fullness or bloating in the upper left abdomen due to an enlarged spleen

• Fatigue

In addition to a complete blood count (CBC), bone marrow biopsies and cytogenetic analysis tests are used to diagnose polycythemia vera. A mutation or change in the body’s JAK 2 gene is believed to be the major cause of PV. A sample of blood is tested for the level of erythropoietin, a hormone that stimulates new red blood cells.

Treatment options

  • Jakafi (ruxolitinib):  Another option to treat PV is a medicine called Jakafi.  It is the first and only FDA-approved treatment for people with PV who have already taken a medicine called hydroxyurea and it did not work well enough or was not tolerated.  For more information click here.
  • Phlebotomy: Taking blood out of your veins is usually the first treatment option. This procedure reduces the number of blood cells and decreases your blood volume, making it easier for your blood to function properly.
  • Hydroxyurea (HU): This has been widely used in the treatment of PV patients as an effective myelosuppressive agent. HU reduces the risk of thrombosis.
  • Low-dose Aspirin: Aspirin prevents platelets from sticking together which helps prevent clotting.
  • Pegalated Interferon: Some PV patients have had success with this treatment. It is currently in clinical trials.

Please consult with your physician about all available treatments to determine the best course of action for you.

Visit the clinical trials page for updates on all drugs currently being tested for the treatment of PV.