What Is the Prognosis of Polycythemia Vera?


Quick Answer

The prognosis of polycythemia vera is fair because the disease is associated with a shortened life span and an average survival time between eight and 15 years, according to The Merck Manual. The leading cause of death is thrombosis, followed by complications from myelofibrosis and the development of leukemia.

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Polycythemia vera is a condition in which all blood cell lines increase in number, including red blood cells, white blood cells and platelets, explains The Merck Manual. This leads to hyperviscosity of the blood and an increased risk of thrombus formation. A thrombus can form in any blood vessel and can cause a stroke, transient ischemic attack, deep venous thrombosis, myocardial infarction, retinal artery or vein occlusion, splenic infarction, or Budd-Chiari syndrome.

The mainstay treatment of polycythemia vera is phlebotomy and regular hematocrit monitoring, according to The Merck Manual. The threshold for undergoing phlebotomy is a hematocrit count of greater than 45 percent in men and 42 percent in women. Initial sessions involve the removal of 300 to 500 milliliters of blood every other day. Less blood is removed in the elderly and patients with heart conditions and cerebrovascular disease.

When hematocrit levels fall below the threshold, phlebotomy is withheld and hematocrit levels are monitored monthly, states The Merck Manual. Other treatment includes myelosuppressive therapy and medication, such as aspirin, hydroxyurea, interferon alfa-2b, JAK2 pathway inhibitors and radioactive phosphorus.

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