What is polycythemia vera?

Polycythemia vera (PV) is a condition that causes your bone marrow to produce too many red blood cells (RBCs). RBCs carry oxygen throughout the body. Too many white blood cells (WBCs) and platelets may also be produced. The extra blood cells make your blood thicker than normal. Blood that is too thick cannot flow easily, so less oxygen is delivered to your body's tissues. Left untreated, PV is life-threatening. PV is usually caused by a gene mutation (change). Your risk for PV increases if you are male or older than 50 years.

What are the signs and symptoms of PV?

Signs and symptoms develop slowly, over many years. You may have any of the following:

• Chest pain or discomfort
• Fatigue, weakness, or weight loss without trying
• Shortness of breath, or trouble breathing when you lie down
• Pressure on the left side of your abdomen, abdominal pain, or diarrhea
• Bulging veins, or blue or purple skin on your face or mucus membranes
• Itching that may be intense, or numbness or burning pain in your feet or hands
• High blood pressure, headaches, dizziness, or blurred vision
• Blood clots, bruising, and bleeding problems
• Pain and swelling in a joint, usually in a big toe, or bone pain

How is PV diagnosed?

PV is often found during tests for other conditions, or to find the cause of symptoms such as shortness of breath. Your healthcare provider will ask about your symptoms and when they started. Tell him about all medicines you are taking. Also tell him about any medical conditions you have. You may need any of the following:

• Blood tests are used to find the number of RBCs, WBCs, and platelets you have. The tests may also be used to check your vitamin B12, uric acid, and erythropoietin (EPO) levels. EPO is a hormone that tells bone marrow to create new blood cells. You may also need a blood test to check for the gene mutation that causes PV.
• A blood oxygen level test may be needed. PV can cause the level to be lower than normal if RBCs cannot deliver oxygen correctly.
• Bone marrow biopsy or aspiration is a procedure used to take a sample of bone marrow to be tested for the amount of iron it contains. The sample may also show if you have healthy bone marrow or if your bone marrow makes a normal amount of blood cells.

How is PV treated?

PV cannot be cured. It will always need to be managed. The goal of treatment is improve symptoms and reduce the risk for problems such as blood clots.

• Phlebotomy is a procedure used to remove blood from your body. About a pint of blood is removed during a session. The procedure lowers the number of RBCs and thins the blood. Phlebotomy may be done every few days to every few months.
• Medicines may be given to thin your blood. This will help reduce blood clots. Medicines may also be given to reduce itching or uric acid, or to control stomach acid. Medicine is sometimes used to make bone marrow create fewer RBCs.
• Aspirin can help thin your blood, and relive bone pain and burning in your hands or feet. Aspirin can cause stomach bleeding in some people. Only take aspirin if directed by your healthcare provider. Do not take more than the recommended amount.
• Radiation is a procedure used to stop bone marrow cells from producing too many RBCs.

Treatment options

The following list of medications are in some way related to or used in the treatment of this condition.

• Jakafi
• hydroxyurea
• Besremi
• ruxolitinib
• ropeginterferon alfa-2b

View more treatment options

What can I do to prevent blood clots?

• Do not smoke. Nicotine and other chemicals in cigarettes and cigars can increase your risk for blood clots and cause lung damage. Ask your healthcare provider for information if you currently smoke and need help to quit. E-cigarettes or smokeless tobacco still contain nicotine. Talk to your healthcare provider before you use these products.
• Help keep your blood flowing. Wear support stockings as directed. Support stockings are tight and help push blood up and out of your leg veins. Elevate your feet when you sit. This will help prevent blood from pooling in your leg veins.
• Exercise as directed. Exercise such as walking helps improve blood flow and prevents blood clots. Ask your healthcare provider which exercises are best for you. Stop if you feel any chest pain or shortness of breath.
• Drink liquids as directed. Liquids help keep your blood thin. Your healthcare provider may recommend that you drink at least 3 liters (12 cups) of liquid each day. Ask which liquids are best for you.

What can I do to manage my symptoms?

• Prevent bleeding and bruising. PV or blood thinners used to manage it can increase your risk for heavy bleeding and easy bruising. Use an electric razor and soft toothbrush. Floss your teeth gently. Do not play contact sports, such as football. These sports increase the risk for bruising. Get care immediately if you are injured. Ask about other ways to prevent bleeding and bruising. Tell all healthcare providers that you have PV or are taking blood thinners.
• Protect your hands and feet. Wear gloves outside if the temperature is low. Check your hands and feet for sores caused by blood circulation problems. You may not feel the sores because of the low blood flow.
• Avoid high temperatures. Do not take hot baths or sit in hot tubs. The heat can increase symptoms such as facial flushing and skin itching. Protect your skin when you are outside in hot weather. Do not use a tanning bed or sun lamp. These can damage your skin.
• Take cool baths to relive itching. Itching can increase after a hot bath. Cool water may help relive itching. You may want to use oatmeal, corn starch, or a mild moisturizing soap to relieve dryness or itching. Lotion may also help relieve dry skin.

Call 911 for any of the following:

• You have any of the following signs of a heart attack:
  • Squeezing, pressure, or pain in your chest
  • You may also have any of the following:
    • Discomfort or pain in your back, neck, jaw, stomach, or arm
    • Shortness of breath
    • Nausea or vomiting
    • Lightheadedness or a sudden cold sweat
• You have any of the following signs of a stroke:
  • Numbness or drooping on one side of your face
  • Weakness in an arm or leg
  • Confusion or difficulty speaking
  • Dizziness, a severe headache, or vision loss
• You have a severe headache or a seizure.
• You cough up blood.

When should I seek immediate care?

• Your arm or leg feels warm, tender, and painful. It may look swollen and red.
• You have new or worsening symptoms.
• You have heavy bleeding that does not stop after 20 minutes of applied pressure.

When should I contact my healthcare provider?

• Your nose or gums bleed.
• You see blood in your urine.
• You have a sore or skin changes on your hands or feet, or under your compression stocking.
• You have more bruises than usual.
• You have questions or concerns about your condition or care.

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