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.
Polycythemia vera is rare. It usually develops slowly, and you might have it for years without knowing. Often the condition is found during a blood test done for another reason.
Without treatment, polycythemia vera can be life-threatening. But proper medical care can help ease signs, symptoms and complications of this disease.
Many people with polycythemia vera don't have noticeable signs or symptoms. Some people might develop vague symptoms such as headache, dizziness, fatigue and blurred vision.
More-specific symptoms of polycythemia vera include:
Make an appointment with your doctor if you have signs or symptoms of polycythemia vera.
Polycythemia vera occurs when a mutation in a gene causes a problem with blood cell production. Normally, your body regulates the number of each of the three types of blood cells you have — red blood cells, white blood cells and platelets. But in polycythemia vera, your bone marrow makes too many of some of these blood cells.
The cause of the gene mutation in polycythemia vera is unknown, but it's generally not inherited from your parents.
Polycythemia vera can occur at any age, but it's more common in adults between 50 and 75. Men are more likely to get polycythemia vera, but women tend to get the disease at younger ages.
Possible complications of polycythemia vera include:
Your doctor will take a detailed medical history and perform a physical exam.
If you have polycythemia vera, blood tests might reveal:
If your doctor suspects that you have polycythemia vera, he or she might recommend collecting a sample of your bone marrow through a bone marrow aspiration or biopsy.
A bone marrow biopsy involves taking a sample of solid bone marrow material. A bone marrow aspiration is usually done at the same time. During an aspiration, your doctor withdraws a sample of the liquid portion of your marrow.
If you have polycythemia vera, analysis of your bone marrow or blood might show the gene mutation that's associated with the disease.
In a bone marrow aspiration, a doctor or nurse uses a thin needle to remove a small amount of liquid bone marrow, usually from a spot in the back of your hipbone (pelvis). A bone marrow biopsy is often done at the same time. This second procedure removes a small piece of bone tissue and the enclosed marrow.
There's no cure for polycythemia vera. Treatment focuses on reducing your risk of complications. These treatments may also ease your symptoms.
Treatment to reduce symptoms might include:
Therapy to reduce itching. If you have bothersome itching, your doctor may prescribe medication, such as antihistamines, or recommend ultraviolet light treatment to relieve your discomfort.
Medications that are normally used to treat depression, called selective serotonin reuptake inhibitors (SSRIs), helped relieve itching in clinical trials. Examples of SSRIs include paroxetine (Brisdelle, Paxil, Pexeva, others) or fluoxetine (Prozac, Sarafem, Selfemra, others).
Treatments to reduce the amount of red blood cells include:
Medication to decrease blood cells. If phlebotomy alone doesn't help, medications that suppress your bone marrow's ability to produce blood cells, such as hydroxyurea (Droxia, Hydrea), might be used.
Interferon alfa-2b (Intron A) may be used to stimulate your immune system to fight the overproduction of red blood cells. It might help people who don't respond well to hydroxyurea.
Medication to destroy cancer cells. Ruxolitinib (Jakafi) is approved by the Food and Drug Administration to treat people with polycythemia vera who don't respond to or can't take hydroxyurea. It helps your immune system destroy cancer cells, and can improve some polycythemia vera symptoms.
Busulfan (Busulfex, Myleran) is another medication that destroys cancer cells that may be prescribed if other treatments aren't working well.
Researchers are studying drugs that inhibit a gene linked to polycythemia vera and other treatments.
Your doctor will also likely prescribe medications to control risk factors for heart and blood vessel disease, including high blood pressure, diabetes and abnormal cholesterol.
You'll need to see your doctor on an ongoing basis to monitor how well your current treatment is working. Ask your doctor how often you need to schedule follow-up appointments.
You can take steps to help yourself feel better if you've been diagnosed with polycythemia vera. Try to:
Be good to your skin. To reduce itching, bathe in cool water, use a gentle cleanser and pat your skin dry. Adding starch, such as cornstarch, to your bath might help. Avoid hot tubs, heated whirlpools, and hot showers or baths.
Try not to scratch, as it can damage your skin and increase the risk of infection. Use lotion to keep your skin moist.
You're likely to start by seeing your primary care physician. If you're diagnosed with polycythemia vera, you might be referred to a doctor who specializes in blood conditions (hematologist).
Here's some information to help you get ready for your appointment.
Make a list of:
For polycythemia vera, questions to ask your doctor include:
Don't hesitate to ask other questions you think of during the appointment. Take a family member or friend along, if possible, to help you remember the information you're given.
Your doctor is likely to ask you questions, including: