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What Is Polycythemia Vera?

Polycythemia vera is a rare blood cancer. It belongs to a wider group of blood cancers called myeloproliferative neoplasms, which cause the blood cells developed by the bone marrow to not function well. 

Polycythemia vera leads to the overproduction of red blood cells, and also often causes an affected person's platelet and white blood cell count to be too high.

While the cause of the cancer is not yet understood very well, we do know that most people who develop it do not have a family history of PV, and we also know that a majority of those affected have a mutation of the JAK2 gene, which almost certainly plays a role in the onset of the condition. Polycythemia vera is more common in women than in men, and it usually doesn't occur before 40 years of age. Research suggests that around three in 100,000 are affected by the cancer across the world. 

What Are The Symptoms Of Polycythemia Vera?

Since Polycythemia vera is a slow-growing cancer, it is possible to have the condition for years before you begin noticing symptoms. Because the excess red blood cells seen in Polycythemia vera lead to very thick blood, proper blood flow eventually becomes difficult, which can cause symptoms like:

  • Blue-tinted skin. 
  • Shortness of breath.
  • Dizziness. 
  • Fatigue. 
  • Headache. 
  • Easy bleeding. 
  • Itching. 
  • A red face. 
  • Vision changes, like blind spots or blurred vision. 
  • Excessive sweating.
  • An enlarged spleen and an associated full feeling in the upper abdomen, on the left side. 

Individually, these symptoms are non-specific and easy to dismiss. When put together, they may tell you that something is wrong — but not also what. Polycythemia vera is, as such, just one of many conditions that demonstrate the importance of seeking medical attention in the face of worrying but not terribly specific symptoms. 

Polycythemia Vera Diagnosis And Treatment

A complete blood count, bone marrow biopsy, testing for JAK2 gene mutations, and a whole host of other tests are used in the diagnosis of Polycythemia vera. 

There is no cure for Polycythemia vera at present, and treatment is aimed at making your blood less thick and preventing clotting issues. Treatments you can expect include:

  • Phlebotomy, or the removal of blood, to reduce your blood's thickness. This procedure may be carried out on a weekly basis. 
  • Aspirin, to reduce the patient's risk of thrombosis.
  • If your platelet count is too high, Anagrelide to reduce platelet production. 
  • Myelosuppressive drugs such as Hydroxyurea and Ruxolitinib, to keep your red blood cell and platelet production in check. 
  • Antihistamines or light therapy to help reduce the itching some people with PV suffer from. 

Because complications of Polycythemia vera include leukemia, gout, heart failure, thrombosis, myelofibrosis, and internal bleeding, your health will need to be monitored closely. 

While Polycythemia vera isn't curable, and research does show that the life expectancy of Polycythemia vera patients is lower than that of the general population (especially if the disease is diagnosed before age 50), you may be interested to hear that there is a 65 percent 15-year survival rate.

Many patients live far longer than that, as well. Polycythemia vera is a very slow-growing cancer, and you have an excellent chance of living for a very long time as long as you receive appropriate treatment. 

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