Patient education: Polycythemia vera (The Basics)

What is polycythemia vera? — 

Polycythemia vera, or "PV," is a condition that affects bone marrow. Bone marrow is the tissue in the center of the bones that makes blood cells.

People with PV make too many red blood cells. (These are the cells that carry oxygen to the body.) They might also have higher-than-normal levels of:

●White blood cells – These cells fight infections.

●Platelets – These help blood to clot, so you stop bleeding after getting a cut or injury.

What are the symptoms of PV? — 

People with PV might have:

●Headaches

●Weakness or dizziness

●A lot of sweating

●Itchiness, especially when rubbing their skin after a warm bath or shower

●Painful, swollen joints, especially in the big toe, ankle, or knee (called "gout")

●Burning pain in their hands and feet, which can also turn red, white, or blue

●Dangerous blood clots

●Vision problems, such as seeing dark spots, sparks, or flashes

●Stomach problems, such as pain in the upper belly

Are there tests for PV? — 

Yes. Your doctor or nurse will do an exam and order different blood tests.

You might also need a bone marrow biopsy. For this test, a doctor takes a very small sample of your bone marrow. Then, another doctor looks at the cells under a microscope.

How is PV treated? — 

Treatments include:

●Removing blood from your body – This is called "phlebotomy." It is similar to what happens when giving blood.

●Low-dose aspirin – This helps prevent blood clots.

●Medicines to keep your bone marrow from making too many red blood cells – These include hydroxyurea (brand names: Droxia, Hydrea), ropeginterferon alfa-2b (brand name: Besremi), ruxolitinib (brand name: Jakafi), busulfan (brand name: Myleran, Busulfex), and others.

Your doctor might also prescribe medicines or other treatments to help with itching.

During treatment, you will see your doctor regularly and get blood tests. This can help them see if treatment is working.

What can I do on my own? — 

You should:

●Manage itching – It might help to:

•Avoid very hot baths or showers. Hot water can make itching worse.

•Dry your skin by patting it with a towel. Do not rub your skin.

•Use moisturizing creams or lotions to help keep your skin from getting too dry.

●Lower your risk of heart disease – People with PV are at risk for blood clots. This can lead to serious problems such as heart attack. To lower this risk, you can:

•Quit smoking, if you smoke – Your doctor or nurse can help.

•Eat a healthy diet – Eat lots of fruits, vegetables, and low-fat dairy products. Don't eat a lot of meats, sweets, or refined grains.

•Get regular physical activity – Experts recommend trying to be active for at least 30 minutes a day on most days of the week. Even gentle forms of exercise, like walking or gardening, are good for your health.

•Keep your blood pressure and cholesterol managed – If your doctor prescribed medicines to help with this, follow the instructions for taking them.

Tell your doctor if you have any new symptoms or problems during treatment.

What else should I know? — 

Getting treated for PV involves making many choices, such as what treatment to have and when.

Always tell your doctors and nurses how you feel about a treatment. Any time you are offered a treatment, ask:

●What are the benefits of this treatment? Is it likely to help me live longer? Will it reduce or prevent symptoms?

●What are the downsides to this treatment?

●Are there other options besides this treatment?

●What happens if I do not have this treatment?

When should I call the doctor? — 

Call for emergency help right away (in the US and Canada, call 9-1-1) if you:

●Have signs of stroke, like sudden:

•Numbness or weakness of the face, arm, or leg, especially on 1 side of the body

•Confusion, or trouble speaking or understanding

•Trouble seeing in 1 or both eyes

•Trouble walking, dizziness, or loss of balance or coordination

•Severe headache with no known cause

●Have signs of a heart attack, like:

•Severe chest pain, pressure, or discomfort with:

-Trouble breathing, sweating, nausea, or cold and clammy skin

-Pain in your arms, back, or jaw

-Worse pain with activity like walking up stairs

•Fast or irregular heartbeat

•Feeling dizzy, faint, or weak

Call your doctor or nurse for advice if:

●You have new or worse swelling in your arm or leg.

●Your arm or leg becomes numb or very painful to touch.

●Your leg hurts when you walk, or your arm hurts when you move it.

●Your arm or leg turns blue or gray.

●You have discomfort taking a deep breath.

More on this topic

Patient education: Complete blood count (CBC) (The Basics)
Patient education: Hemoglobin and hematocrit tests (The Basics)
Patient education: Itchy skin (The Basics)
Patient education: Bone marrow aspiration and biopsy (The Basics)
Patient education: Therapeutic phlebotomy (The Basics)