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Chronic Myeloproliferative Disorders Treatment (PDQ®)

Patient Version
Last Modified: 09/27/2011

Polycythemia Vera

Key Points for This Section


Polycythemia vera is a disease in which too many red blood cells are made in the bone marrow.

In polycythemia vera, the blood becomes thickened with too many red blood cells. The number of white blood cells and platelets may also increase. These extra blood cells may collect in the spleen and cause it to swell. The increased number of red blood cells or platelets in the blood can cause bleeding problems and make clots form in blood vessels. This can increase the risk of stroke or heart attack. In patients who are older than 65 years or who have a history of blood clots, the risk of stroke or heart attack is higher. Patients also have an increased risk of developing acute myeloid leukemia or primary myelofibrosis.

Possible signs of polycythemia vera include headaches and a feeling of fullness below the ribs on the left side.

Polycythemia vera often does not cause early symptoms. It is sometimes found during a routine blood test. Symptoms may occur as the number of blood cells increases. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • A feeling of pressure or fullness below the ribs on the left side.
  • Headaches.
  • Double vision or seeing dark or blind spots that come and go.
  • Itching all over the body, especially after being in warm or hot water.
  • Reddened face that looks like a blush or sunburn.
  • Weakness.
  • Dizziness.
  • Weight loss for no known reason.

Special blood tests are used to diagnose polycythemia vera.

In addition to a complete blood count, bone marrow aspiration and biopsy, and cytogenetic analysis, a serum erythropoietin test is used to diagnose polycythemia vera. In this test, a sample of blood is checked for the level of erythropoietin (a hormone that stimulates new red blood cells to be made).



Glossary Terms

acute myeloid leukemia (uh-KYOOT MY-eh-loyd loo-KEE-mee-uh)
An aggressive (fast-growing) disease in which too many myeloblasts (immature white blood cells that are not lymphoblasts) are found in the bone marrow and blood. Also called acute myeloblastic leukemia, acute myelogenous leukemia, acute nonlymphocytic leukemia, AML, and ANLL.
blood (blud)
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 clot (blud klot)
A mass of blood that forms when blood platelets, proteins, and cells stick together. When a blood clot is attached to the wall of a blood vessel, it is called a thrombus. When it moves through the bloodstream and blocks the flow of blood in another part of the body, it is called an embolus.
blood vessel (blud VEH-sel)
A tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.
bone marrow aspiration (bone MAYR-oh AS-pih-RAY-shun)
A procedure in which a small sample of bone marrow is removed, usually from the hip bone, breastbone, or thigh bone. A small area of skin and the surface of the bone underneath are numbed with an anesthetic. Then, a special wide needle is pushed into the bone. A sample of liquid bone marrow is removed with a syringe attached to the needle. The bone marrow is sent to a laboratory to be looked at under a microscope. This procedure may be done at the same time as a bone marrow biopsy.
bone marrow biopsy (bone MAYR-oh BY-op-see)
A procedure in which a small sample of bone with bone marrow inside it is removed, usually from the hip bone. A small area of skin and the surface of the bone underneath are numbed with an anesthetic. Then, a special, wide needle is pushed into the bone and rotated to remove a sample of bone with the bone marrow inside it. The sample is sent to a laboratory to be looked at under a microscope. This procedure may be done at the same time as a bone marrow aspiration.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
complete blood count (kum-PLEET blud kownt)
A test to check the number of red blood cells, white blood cells, and platelets in a sample of blood. Also called blood cell count and CBC.
cytogenetics (SY-toh-jeh-NEH-tix)
The study of chromosomes and chromosomal abnormalities.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
erythropoietin (eh-RITH-roh-POY-eh-tin)
A substance that is naturally produced by the kidneys, and that stimulates the bone marrow to make red blood cells. When erythropoietin is made in the laboratory, it is called epoetin alfa or epoetin beta.
hormone (HOR-mone)
One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.
platelet (PLAYT-let)
A tiny piece of a cell found in the blood that breaks off from a large cell found in the bone marrow. Platelets help wounds heal and prevent bleeding by forming blood clots. Also called thrombocyte.
polycythemia vera (PAH-lee-sy-THEE-mee-uh VAYR-uh)
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. The extra blood cells may collect in the spleen and cause it to become enlarged. They may also cause bleeding problems and make clots form in blood vessels.
primary myelofibrosis (PRY-mayr-ee MY-eh-loh-fy-BROH-sis)
A progressive, chronic disease in which the bone marrow is replaced by fibrous tissue and blood is made in organs such as the liver and the spleen, instead of in the bone marrow. This disease is marked by an enlarged spleen and progressive anemia. Also called agnogenic myeloid metaplasia, chronic idiopathic myelofibrosis, idiopathic myelofibrosis, and myelosclerosis with myeloid metaplasia.
red blood cell (red blud sel)
A cell that carries oxygen to all parts of the body. Also called erythrocyte and RBC.
serum (SEER-um)
The clear liquid part of the blood that remains after blood cells and clotting proteins have been removed.
spleen (spleen)
An organ that is part of the lymphatic system. The spleen makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach.
stroke (stroke)
In medicine, a loss of blood flow to part of the brain, which damages brain tissue. Strokes are caused by blood clots and broken blood vessels in the brain. Symptoms include dizziness, numbness, weakness on one side of the body, and problems with talking, writing, or understanding language. The risk of stroke is increased by high blood pressure, older age, smoking, diabetes, high cholesterol, heart disease, atherosclerosis (a build-up of fatty material and plaque inside the coronary arteries), and a family history of stroke.
symptom (SIMP-tum)
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
white blood cell (hwite blud sel)
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. Also called leukocyte and WBC.