Polycythemia vera is a myeloproliferative neoplasm of the blood-producing cells of the bone marrow that results in overproduction of all types of blood cells. Polycythemia vera is due to mutations in the Janus kinase 2 (JAK2) gene, which produces a protein (enzyme) that stimulates excessive production of blood cells. People may feel tired and weak, light-headed, or short of breath, or develop symptoms caused by blood clots. Blood tests are done for diagnosis. Phlebotomy is done to remove excess red blood cells, and some people take aspirin and sometimes other drugs. In polycythemia vera, the excess of red blood cells increases the volume of blood and makes it thicker, so that it flows less easily through small blood vessels. Polycythemia vera occurs in about 2 in every 100,000 people. The average age at which the disorder is diagnosed is 60, but it also occurs in people younger than 40. More men than women develop polycythemia vera over age 60, but more women than men develop the disease under age 40. More than 95% of people with polycythemia vera have a mutation in the JAK2 gene. This mutation causes excess blood cell production. In addition, mutations in the calreticulin gene (CALR) and other genes have been found in some people with polycythemia vera. These mutations lead to sustained activation of JAK2 kinase, the enzyme that causes excess red blood cell production. Often, people with polycythemia vera have no symptoms for years. The earliest symptoms usually are
Vision may be distorted, and people may have blind spots or see flashes of light (ocular migraine). People may have bleeding from the digestive tract or gums and more bleeding than would be expected from small cuts. A
blood clot sometimes causes the first symptoms. The increase in red blood cells in polycythemia vera makes the blood thicker and more likely to clot more than normal. A clot may form in almost any blood vessel, including those of the arms, legs (causing
deep vein thrombosis
Deep Vein Thrombosis (DVT) Deep vein thrombosis is the formation of blood clots (thrombi) in the deep veins, usually in the legs. Blood clots may form in veins if the vein is injured, a disorder causes the blood to clot... read more
Polycythemia vera may be discovered through a blood count done for another reason, even before people have any symptoms. The number of red blood cells, the level of the protein that carries oxygen in red blood cells (hemoglobin) and the percentage of red blood cells in the total blood volume (the hematocrit) are abnormally high. The number of platelets and white blood cells may also be increased. Blood levels of erythropoietin, a hormone that stimulates the bone marrow to produce red blood cells, also may be measured. Levels of erythropoietin are usually, extremely low in polycythemia vera, and they are often, but not always, normal or high in secondary erythrocytosis. People are tested for JAK 2 gene mutations to confirm the diagnosis of polycythemia vera. If those mutations are not found, doctors look for CALR and sometimes other mutations, which are also helpful in confirming the diagnosis.
Aspirin can help relieve symptoms related to the high platelet counts, such as migraines that affect vision and burning pain and redness of the hands and feet. However, aspirin has not been proven to decrease the risk of blood clots in polycythemia vera and does not benefit people who do not have symptoms People who continue to have symptoms after having phlebotomy may need other treatments. For such people, doctors may use ruxolitinib, a drug that inhibits the activity of JAK2 , or other drugs, including pegylated interferon alfa-2b, anagrelide, or hydroxyurea. The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource. CLICK HERE FOR THE PROFESSIONAL VERSION  Copyright © 2022 Merck & Co., Inc., Rahway, NJ, USA and its affiliates. All rights reserved. Does polycythemia cause high white blood count?Polycythaemia vera (PV)
The affected bone marrow cells can also develop into other cells found in the blood, which means that people with PV may also have abnormally high numbers of both platelets and white bloods cells.
What does polycythemia increase?Polycythemia vera is a rare blood disorder in which there is an increase in all blood cells, particularly red blood cells. The increase in blood cells makes your blood thicker. This can lead to strokes or tissue and organ damage.
What is an abnormal increase in white blood cells?Leukocytosis, or high white blood cell count, can indicate a range of conditions, including infections, inflammation, injury and immune system disorders. A complete blood count (CBC) is usually performed to check for leukocytosis. Treating the underlying condition usually reduces your white blood cell count.
What cells are increased in polycythemia vera?Polycythemia vera (pol-e-sy-THEE-me-uh VEER-uh) is a type of blood cancer. It causes your bone marrow to make too many red blood cells. These excess cells thicken your blood, slowing its flow, which may cause serious problems, such as blood clots.
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Is polycythemia is an abnormal increase in the number of white blood cells in the blood?
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