Overview

Polycythemia vera (pol-e-sy-THEE-me-uh VEER-uh) is a type of blood cancer. It causes your bone marrow to make too many red blood cells. These excess cells thicken your blood, slowing its flow, which may cause serious problems, such as blood clots.

Polycythemia vera is rare. It usually develops slowly, and you might have it for years without knowing. Often the condition is found during a blood test done for another reason.

Without treatment, polycythemia vera can be life-threatening. But proper medical care can help ease signs, symptoms and complications of this disease.

Symptoms

Many people with polycythemia vera don't have noticeable signs or symptoms. Some people might develop vague symptoms such as headache, dizziness, fatigue and blurred vision.

More-specific symptoms of polycythemia vera include:

  • Itchiness, especially after a warm bath or shower
  • Numbness, tingling, burning, or weakness in your hands, feet, arms or legs
  • A feeling of fullness soon after eating and bloating or pain in your left upper abdomen due to an enlarged spleen
  • Unusual bleeding, such as a nosebleed or bleeding gums
  • Painful swelling of one joint, often the big toe
  • Shortness of breath and difficulty breathing when lying down

When to see a doctor

Make an appointment with your doctor if you have signs or symptoms of polycythemia vera.

Causes

Polycythemia vera occurs when a mutation in a gene causes a problem with blood cell production. Normally, your body regulates the number of each of the three types of blood cells you have — red blood cells, white blood cells and platelets. But in polycythemia vera, your bone marrow makes too many of some of these blood cells.

The cause of the gene mutation in polycythemia vera is unknown, but it's generally not inherited from your parents.

Risk factors

Polycythemia vera can occur at any age, but it's more common in adults between 50 and 75. Men are more likely to get polycythemia vera, but women tend to get the disease at younger ages.

Complications

Possible complications of polycythemia vera include:

  • Blood clots. Increased blood thickness and decreased blood flow, as well as abnormalities in your platelets, raise your risk of blood clots. Blood clots can cause a stroke, a heart attack, or a blockage in an artery in your lungs or a vein deep within a leg muscle or in the abdomen.
  • Enlarged spleen. Your spleen helps your body fight infection and filter unwanted material, such as old or damaged blood cells. The increased number of blood cells caused by polycythemia vera makes your spleen work harder than normal, which causes it to enlarge.
  • Problems due to high levels of red blood cells. Too many red blood cells can lead to a number of other complications, including open sores on the inside lining of your stomach, upper small intestine or esophagus (peptic ulcers) and inflammation in your joints (gout).
  • Other blood disorders. In rare cases, polycythemia vera can lead to other blood diseases, including a progressive disorder in which bone marrow is replaced with scar tissue, a condition in which stem cells don't mature or function properly, or cancer of the blood and bone marrow (acute leukemia).

Jan. 23, 2020
  1. Ferri FF. Polycythemia vera. In: Ferri's Clinical Advisor 2020. Elsevier; 2020. https://www.clinicalkey.com. Accessed Dec. 7, 2019.
  2. Polycythemia vera. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health-topics/polycythemia-vera. Accessed Dec. 7, 2019.
  3. Goldman L, et al., eds. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In: Goldman-Cecil Medicine. 26th ed. Elsevier; 2020. https://www.clinicalkey.com. Accessed Dec. 8, 2019.
  4. AskMayoExpert. Polycythemia vera (adult). Mayo Clinic. 2019.
  5. Cuthbert D, et al. Polycythemia vera-associated complications: Pathogenesis, clinical manifestations, and effects on outcomes. Journal of Blood Medicine. 2019; doi:10.2147/JBM.S189922.
  6. Tefferi A, et al. Polycythemia vera treatment algorithm 2018. Blood Cancer Journal. 2018; doi:10.1038/s41408-017-0042-7.
  7. Tefferi A. Prognosis and treatment of polycythemia vera. https://www.uptodate.com/contents/search. Accessed Dec. 7, 2019.
  8. Griesshammer M, et al. Thromboembolic events in polycythemia vera. Annals of Hematology. 2019; doi:10.1007/s00277-019-03625-x.
  9. Spivak JL. Polycythemia vera. Current Treatment Options in Oncology. 2018; doi:10.1007/s11864-018-0529-x.
  10. Van de Ree-Pellikaan C, et al. Treatment strategies for polycythemia vera: Observations in a Dutch "real-world" cohort study. European Journal of Haematology. 2019; doi:10.1111/ejh.13291.
  11. Ferrari A, et al. Clinical outcomes under hydroxyurea treatment in polycythemia vera: A systematic review and meta-analysis. Haematologica. 2019; doi:10.3324/haematol.2019.221234.