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Polycythemia Vera

A disease in which there are too many red blood cells in the bone marrow and blood, causing the blood to thicken. The number of white blood cells and platelets may also increase.

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

Polycythemia Vera

Polycythemia vera (POL-e-si-THEE-me-ah VAY-rah or VE-rah), or PV, is a rare blood disease in which your body makes too many red blood cells.

The extra red blood cells make your blood thicker than normal. As a result, blood clots can form more easily. These clots can block blood flow through your arteries and veins, which can cause a heart attack or stroke.

Thicker blood also doesn't flow as quickly to your body as normal blood. Slowed blood flow prevents your organs from getting enough oxygen, which can cause serious problems, such as angina (an-JI-nuh or AN-juh-nuh) and heart failure. (Angina is chest pain or discomfort.)

Overview

Red blood cells carry oxygen to all parts of your body. They also remove carbon dioxide (a waste product) from your body's cells and carry it to the lungs to be exhaled.

Red blood cells are made in your bone marrow—a sponge-like tissue inside the bones....Read more about Polycythemia Vera
NIH - National Heart, Lung, and Blood Institute

What works? Research summarized

Evidence reviews

Antiplatelet drugs for preventing arterial and venous thrombotic events in patients with polycythaemia vera or essential thrombocythaemia

Low‐dose aspirin as an antiplatelet therapy is the drug of choice for preventing cardiovascular events, but the potential risk of bleeding has limited its use in myeloproliferative neoplasms in the past. Evidence from this review of 630 people in two trials suggests that, in patients with polycythaemia vera and with no clear indication or contraindication to aspirin therapy, low‐dose aspirin may reduce the risk of thrombotic and all‐cause mortality. No data were provided on mortality from bleeding episodes. No studies in participants with essential thrombocythaemia and with antiplatelet therapy other than aspirin have been published.

The management of malignant thrombocytosis in Philadelphia chromosome-negative myeloproliferative disease: guideline recommendations

Bibliographic details: Matthews J H, Smith C A, Herst J, Lee D, Imrie K, Hematology Disease Site Group.  The management of malignant thrombocytosis in Philadelphia chromosome-negative myeloproliferative disease: guideline recommendations. This paper is produced by Cancer Care Ontario Practice Guidelines Initiative. The series is published on the Internet and regularly updated. To ensure that you are viewing the most up to date version, go to the Cancer Care Ontario website at: https://www.cancercare.on.ca/toolbox/qualityguidelines/ Available from: http://www.cancercare.on.ca/common/pages/UserFile.aspx?fileId=34319

Is JAK2 V617F mutation more than a diagnostic index? A meta-analysis of clinical outcomes in essential thrombocythemia

A systematic review and meta-analysis was carried out to compare the frequency of clinically significant outcomes between JAK2 V617F positive and wild type patients with essential thrombocythemia (ET). JAK2 V617F positivity in patients with ET was associated with a clear increase in the odds of thrombosis [OR=1.83 (95% CI, 1.32-2.53), p<0.0001], and much higher odds of transformation to polycythemia vera [OR=7.67 (95% CI, 2.04-28.87), p=0.0009]. The mean difference of the white blood cell count between JAK2 positive and negative patients was associated with an increased odds ratio for thrombosis (p=0.02). The JAK2 V617F mutation in patients with ET is associated with an increased risk of adverse cardiovascular outcomes via an increase in the leukocyte count.

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Summaries for consumers

Ruxolitinib (Jakavi) for polycythaemia vera: Overview

Ruxolitinib (trade name: Jakavi) has been approved since March 2015 for the treatment of polycythemia vera in adults. It is a treatment option if the drug hydroxyurea is not effective or not tolerated.

Antiplatelet drugs for preventing arterial and venous thrombotic events in patients with polycythaemia vera or essential thrombocythaemia

Low‐dose aspirin as an antiplatelet therapy is the drug of choice for preventing cardiovascular events, but the potential risk of bleeding has limited its use in myeloproliferative neoplasms in the past. Evidence from this review of 630 people in two trials suggests that, in patients with polycythaemia vera and with no clear indication or contraindication to aspirin therapy, low‐dose aspirin may reduce the risk of thrombotic and all‐cause mortality. No data were provided on mortality from bleeding episodes. No studies in participants with essential thrombocythaemia and with antiplatelet therapy other than aspirin have been published.

Chronic Myeloproliferative Neoplasms Treatment (PDQ®): Patient Version

Expert-reviewed information summary about the treatment of chronic myeloproliferative neoplasms.

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Terms to know

Blood
A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
Blood Plasma
The clear, yellowish, fluid part of the blood that carries the blood cells. The proteins that form blood clots are in plasma.
Bone Marrow
The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.
Erythrocytes (Red Blood Cells)
A cell that carries oxygen to all parts of the body.
Genetic
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
Leukocytes (White Blood Cells)
A type of immune cell. Most white blood cells are made in the bone marrow and are found in the blood and lymph tissue. White blood cells help the body fight infections and other diseases. Granulocytes, monocytes, and lymphocytes are white blood cells.
Platelets (Thrombocytes)
A tiny piece of cell that is made by breaking off of a large cell in the bone marrow. Platelets are found in the blood and spleen. They help form blood clots to slow or stop bleeding, and to help wounds heal. Also called thrombocyte.

More about Polycythemia Vera

Photo of an adult

Also called: Cryptogenic polycythemia, Erythremia, Erythrocytosis, Myelopathic polycythemia, Myeloproliferative disorder, Osler disease, Polycythemia rubra vera, Polycythemia with chronic cyanosis, Primary polycythemia, Splenomegalic polycythemia, Vaquez disease

Other terms to know: See all 7
Blood, Blood Plasma, Bone Marrow

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