My friend has polycythemia vera. What exactly is that?
DEAR DR. GOTT: A friend of mine has been diagnosed with polycythemia vera. She is 71 years old and in good health. Fifteen years ago, she had deep vein thrombosis and took warfarin for three months. She now takes an 81-mg aspirin every day, and she took quinine until it was removed from the market.
What is polycythemia vera? What can she expect to happen to her health?
DEAR READER: This is a disorder in which there is an overproduction of red blood cells in the blood and bone marrow. The result is, thicker blood that can't flow as freely as it should through small blood vessels. The liver and spleen can also produce excess blood cells, resulting in bleeding problems, possible clot formation and an enlarged spleen and liver.
A person may not experience any symptoms for years and may be unaware of the condition unless routine laboratory testing is performed. Weakness, fatigue, night sweats, headache and shortness of breath may be present. There may be bleeding from the gums and more profuse bleeding from small cuts than that experienced previously. Vision may be affected, resulting in flashes of light or blind spots. A blood clot may form. A sense of fullness of the abdomen may be apparent. The additional red blood cells also have a connection with kidney stones, gout and stomach ulcers.
Diagnosis can generally be made through laboratory testing. When polycythemia vera is present, the level of protein that carries oxygen in red blood cells and the percentage of red blood cells in the blood will be abnormally high. The number of white blood cells and platelets may also be elevated. When questions regarding diagnosis are present, a test utilizing radioactivity may be performed. Further testing of a specific hormone that stimulates bone marrow to produce red blood cells may be measured. And a sample of bone marrow may be taken for further confirmation.
Treatment will not cure polycythemia vera, but it will control symptoms and decrease the likelihood of complications. One method used is the removal of blood from the body in a manner similar to blood donation. One pint of blood is withdrawn every other day until the levels are normal. Because this process can increase the number of platelets and doesn't have the capability of reducing the size of the liver or spleen, drugs to suppress the production of red blood cells and platelets may be necessary.
With treatment, people live an average of 15 to 20 years following diagnosis. The outlook is not favourable if treatment is refused. Your friend should be under the care of a hematologist, who can provide the best possible care. Even though she had deep venous thrombosis in the past, was placed on an anticoagulant, and hasn't had a recurrence, her physician or specialist should be informed. He or she will want to monitor the situation more carefully to do everything possible so another clot doesn't form now that she has been diagnosed with polycythemia vera.