Health Encyclopedia

Polycythemia vera

Definition

Polycythemia vera is an abnormal increase in blood cells (primarily red blood cells) due to excess production of the cells 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 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 age 40. The exact cause is unknown.

Symptoms

Breathing difficulty when lying down
Dizziness
Fullness in the left upper abdomen
Headache
Itchiness, especially following a warm bath
Red coloration, especially of the face
Shortness of breath
Symptoms of phlebitis

Note: Symptoms are due to increased blood thickness and clotting.

Additional symptoms that may be associated with this disease:

Signs and tests

The health care provider will perform a physical exam. Tests that may be done include:

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

Treatment

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

A method called phlebotomy is used to decrease blood thickness. One pint of blood is removed weekly until the hematocrit level is less than 45, then therapy is continued as needed.

Occasionally, chemotherapy (specifically hydroxyurea) may be given to suppress the bone marrow. Interferon may be given to lower blood counts. A medicine called anegrelide may be given to lower platelet counts.

The use of blood thinners (such as aspirin) is controversial because it may cause stomach bleeding.

Expectations (prognosis)

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. This occurs in a minority of cases.

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.

Complications

Gastric bleeding
Gout
Heart failure
Leukemia
Myelofibrosis
Peptic ulcer disease
Thrombosis (a cause of stroke or heart attack)

Calling your health care provider

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

Review Date:8/28/2007
Reviewed By:Stephen Grund, M.D. Ph.D., Chief of Hematology/Oncology and Director of the George Bray Cancer Center at New Britain General Hospital, New Britain, CT. Review provided by VeriMed Healthcare Network.

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