Polycythemia Vera

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Polycythemia vera is a type of blood cancer known as a myeloproliferative neoplasm. It involves the abnormal development and function of bone marrow cells that produce blood cells, and leads to the overproduction of red blood cells. White blood cells and platelets may also be overproduced. There is currently no cure for polycythemia vera, but the condition can often be managed for many years and the recent FDA approval of Jakafi® is expanding treatment options. In rare cases, polycythemia vera may progress to myelofibrosis (scarring of the bone marrow) or acute myeloid leukemia (AML).

The following is a general overview of the diagnosis and treatment of polycythemia vera. Each person with polycythemia vera is different, and the specific characteristics of your condition will determine how it is managed. The information on this Web site is intended to help educate you about treatment options and to facilitate a shared decision-making process with your treating physician.

Symptoms of Polycythemia Vera

At its early stages, polycythemia vera may not cause any symptoms. Symptoms that may develop as the condition progresses include the following1:

  • A feeling of pressure or fullness below the ribs on the left side.
  • Headaches.
  • Double vision or seeing dark or blind spots that come and go.
  • Itching all over the body, especially after being in warm or hot water.
  • Reddened face that looks like a blush or sunburn.
  • Weakness.
  • Dizziness.
  • Weight loss for no known reason.

Overproduction of blood cells and changes to blood flow increase the risk of serious blood clots in people with polycythemia vera. This can lead to life-threatening conditions such as heart attack, stroke, or pulmonary embolism. Treatment can reduce this risk while also helping to manage bothersome symptoms. Polycythemia vera can also cause pregnancy complications, and women who are pregnant or considering becoming pregnant may wish to talk with their doctor about how to manage their health.

Diagnosis of Polycythemia Vera

Blood tests provide the primary information necessary to diagnosis polycythemia vera. Patients may also undergo a bone marrow examination. Polycythemia vera typically involves a high concentration of red blood cells and the presence of certain gene mutations in blood cells.2 These gene mutations, which involve the Janus kinase 2 (JAK2) gene, are identified in almost all people with polycythemia vera. JAK2 mutations are thought to contribute to the growth of polycythemia vera and some other myeloproliferative neoplasms, but the exact role of this gene continues to be studied.

Another common characteristic of polycythemia vera is lower-than-normal blood levels of a protein known as erythropoietin. People with polycythemia may also have elevated levels of platelets and/or white blood cells.

Treatment of Polycythemia Vera