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POLYCYTHEMIA

General Information

DEFINITION--

    An increase in red blood cells in the body. The disease has 3 forms:

  • Polycythemia vera, which involves overproduction of red blood cells, white blood cells and platelets.
  • Secondary polycythemia (pseudo-polycythemia), which is a complication of diseases or factors other than blood-cell disorders.
  • Stress polycythemia (pseudo-polycythemia), which involves decreased blood plasma.

BODY PARTS INVOLVED--Blood-forming organs: bone marrow; spleen; lymph glands; lymph channels.

SEX OR AGE MOST AFFECTED--Adults of both sexes, but more common in men; usually over age 50.

SIGNS & SYMPTOMS--

    Some patients have no symptoms. Others have any of the following:

  • Fatigue; headache; drowsiness; dizziness.
  • Itching or flushed skin.
  • Enlarged spleen.
  • Unexplained bleeding.

CAUSES

> Polycythemia vera: unknown.

> Secondary polycythemia: congenital heart disease; chronic lung disease; cigarette or cigar smoking; living at high altitude.

> Stress polycythemia: use of diuretic drugs; smoking; dehydration.

RISK INCREASES WITH

  • Smoking.
  • Heart or lung disease.
  • Stress.
  • Family history of polycythemia.

HOW TO PREVENT

Polycythemia vera cannot be prevented at present. > To help prevent secondary polycythemia or stress polycythemia:

    Don't smoke. Avoid dehydration. Obtain medical treatment for heart or lung disease.


What To Expect

DIAGNOSTIC MEASURES--

  • Your own observation of symptoms.
  • Medical history and physical exam by a doctor.
  • Laboratory studies of bone marrow and blood (red-blood-cell count, measurement of hematocrit).
  • X-ray of the kidneys.
  • Radioactive chromium studies.

APPROPRIATE HEALTH CARE

Doctor's treatment. Treatment steps will be individualized depending on your age, disease duration, type of polycythemia, complications and disease activity. > Possible treatment steps to keep the hematocrit range near normal and prevent clotting or hemorrhage are: Phlebotomy (withdrawal of excess blood); radioisotope therapy; and drug therapy. The treatment chosen will depend upon symptoms and response

    to treatment. More than one form of treatment may be needed.

POSSIBLE COMPLICATIONS

  • Clots in veins or arteries.
  • Gout.
  • Stroke.
  • Heart attack.
  • Peptic ulcer.
  • Kidney stones.
  • Leukemia.

PROBABLE OUTCOME--

  • Polycythemia vera is incurable, but symptoms can be controlled. With treatment, average survival ranges from 7-15 years, with some patients living 20 or more years.
  • Other forms of polycythemia can be cured if the causes can be eliminated.

How To Treat

GENERAL MEASURES--

  • Stop smoking. Consult your doctor about recommendations for a cessation program.
  • Adherence to your treatment plan brings about improved survival and well-being.

MEDICATION--Your doctor may prescribe:

  • Aspirin to decrease clotting and reduce the chance of stroke or heart attack may be recommended.
  • Radioactive phosphorus or cytotoxic drugs.
  • Allopurinol for elevated uric acid.
  • Anti-itching medications.
  • H2-receptor or antacids for hyperacidity.

ACTIVITY--After treatment, resume normal activity as soon as possible.

DIET--No special diet. Drink 6 to 8 oz. of fluid every 2 hours to maintain adequate body fluid.


Call Your Doctor If

  • You have symptoms of polycythemia.
  • You have symptoms of complications (refer to specific disorder in this book).
  • New, unexplained symptoms develop. Drugs used in treatment may produce side effects.
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