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Polycythemia vera

 

Polycythemia vera is a bone marrow disease that leads to an abnormal increase in the number of blood cells. The red blood cells are mostly affected.

Alternative Names

Primary polycythemia; Polycythemia rubra vera; Myeloproliferative disorder; Erythremia; Splenomegalic polycythemia; Vaquez's disease; Osler's disease; Polycythemia with chronic cyanosis - Myelopathic polycythemia; Erythrocytosis megalosplenica; Cryptogenic polycythemia

Causes

Polycythemia vera is a disorder of the bone marrow. It mainly causes too many red blood cells to be produced. The numbers of white blood cells and platelets may increase as well..

This is a rare disorder that occurs more often in men than in women. It is not often seen in people under age 40. The problem is often linked to a gene defect called JAK2V617F. The cause of this gene defect is unknown.

Symptoms

  • Trouble breathing when lying down
  • Dizziness
  • Excess bleeding
  • Full feeling in the left upper abdomen (due to enlarged spleen)
  • Headache
  • Itchiness, especially after a warm bath
  • Red skin coloring, especially of the face
  • Shortness of breath
  • Symptoms of blood clots in veins near the skin surface (phlebitis)

Other symptoms that may occur with this disease:

  • Bluish skin color
  • Fatigue
  • Red skin spots
  • Vision problems

Exams and Tests

The health care provider will perform a physical exam. You may also have the following tests:

  • Bone marrow biopsy
  • Complete blood count with differential
  • Comprehensive metabolic panel
  • Erythropoietin level
  • Genetic test for the JAK2V617F mutation
  • Oxygen saturation of the blood
  • Red blood cell mass
  • Vitamin B12 level

This disease may also affect the results of the following tests:

  • ESR
  • Lactate dehydrogenase (LDH)
  • Leukocyte alkaline phosphatase
  • Platelet aggregation test
  • Serum uric acid

Treatment

The goal of treatment is to reduce the thickness of the blood and prevent bleeding and clotting.

A method called phlebotomy is used to decrease blood thickness. One unit of blood (about 1 pint) is removed each week until the number of red blood cells drops. The treatment is continued as needed.

Other treatments may include:

  • Chemotherapy (specifically hydroxyurea) to reduce the number of red blood cells made by the bone marrow. This option may used when the numbers of other blood cell types are also high.
  • Interferon to lower blood counts.
  • Anagrelide to lower platelet counts

Taking aspirin to reduce the risk of blood clots may be an option for some people. However, aspirin increase the risk of stomach bleeding.

Ultraviolet-B light therapy can reduce the severe itching some patients experience.

Outlook (Prognosis)

The disease usually develops slowly. Most patients do not have problems related to the disease after being diagnosed. The condition is often diagnosed before severe symptoms occur.

Possible Complications

When to Contact a Medical Professional

Call your health care provider if symptoms of polycythemia vera develop.

References

Kremyanskaya M, Najfeld V, Mascarenhas J et al. The polycythemias. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Saunders Elsevier; 2012:chap 67.

Tefferi A. Polycythemias. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 169.

Updated: 3/3/2013

Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.


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