Polycythemia vera is an abnormal increase in blood cells (primarily red blood cells) resulting from excess production by the bone marrow.

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, incidence, and risk factors

Polycythemia vera is an acquired disorder of the bone marrow that causes the overproduction of all three blood cell lines: white blood cells, red blood cells, and platelets . It is a rare disease that occurs more frequently in men than women, and rarely in patients under 40 years old. It is not known what causes polycythemia vera. The disease usually develops slowly, and most patients do not experience any problems related to the disease after being diagnosed. However, the abnormal bone marrow cells may begin to grow uncontrollably in some patients leading to acute myelogenous leukemia . Patients with polycythemia vera also have an increased tendency to form blood clots that can result in strokes or heart attacks . Some patients may experience abnormal bleeding because their platelets are abnormal.

Signs and tests

A

CBC with differential

Hematocrit

, elevated

White blood count

, elevated

Platelet count

, elevated

Bone marrow biopsy

Vitamin B-12 level

Chemistry panel

Blood volume, increased

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

Lactate dehydrogenase

Urinalysis

Serum uric acid

TWBC

RBC count

Platelet aggregation test

Leukocyte alkaline phosphatase

Hemoglobin

ESR

Erythropoietin

Treatment

The objective of treatment is to reduce the high blood viscosity (thickness of the blood) due to the increased red blood cell mass and to prevent hemorrhage and thrombosis.

Phlebotomy is one method used to reduce the high blood viscosity. In phlebotomy, 1 unit (pint) of blood is removed weekly until the hematocrit is less than 45, then phlebotomy is continued as necessary. Occasionally, chemotherapy may be given to suppress the bone marrow. The use of anti-platelet therapy (such as aspirin) is controversial because it may cause gastric bleeding. Allopurinol is given for hyperuricemia ( gout ).

Expectations (prognosis)

The median survival is 11 to 15 years. The major cause of death is thrombosis ( blood clots causing strokes or heart attacks ). Polycythemia may also progress and become leukemia .

Complications

Thrombosis (a cause of

stroke and heart attack )

Peptic ulcer disease

Gastric bleeding

Gout

Leukemia

Heart failure

Myelofibrosis

Calling your health care provider

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