Diagnosis

Your doctor will take a detailed medical history and perform a physical exam.

Blood tests

If you have polycythemia vera, blood tests might reveal:

  • More red blood cells than normal and, sometimes, an increase in platelets or white blood cells
  • A greater percentage of red blood cells that make up total blood volume (hematocrit measurement)
  • Elevated levels of the iron-rich protein in red blood cells that carries oxygen (hemoglobin)

Bone marrow aspiration or biopsy

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.

A needle suctioning out liquid bone marrow from hipbone

Bone marrow exam

In a bone marrow aspiration, a health care provider 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.

Specific gene testing

If you have polycythemia vera, analysis of your bone marrow or blood might show the gene mutation that's associated with the disease.


Treatment

There's no cure for polycythemia vera. Treatment focuses on reducing your risk of complications. These treatments may also ease your symptoms.

Blood withdrawals

The most common treatment for polycythemia vera is having frequent blood withdrawals, using a needle in a vein (phlebotomy). It's the same procedure used for donating blood.

This decreases your blood volume and reduces the number of excess blood cells. How often you need to have blood drawn depends on the severity of your condition.

Treatments 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 selective serotonin reuptake inhibitors (SSRIs) include paroxetine (Brisdelle, Paxil, Pexeva, others) or fluoxetine (Prozac, Sarafem, Selfemra, others).

Drugs that reduce the number of red blood cells

If phlebotomy alone doesn't help enough, your doctor may suggest medications that can reduce the number of red blood cells in your bloodstream. Examples include:

  • Hydroxyruea (Droxia, Hydrea)
  • Interferon alfa-2b (Intron A)
  • Ruxolitinib (Jakafi)
  • Busulfan (Busulfex, Myleran)

Heart medications

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.

Your doctor may recommend that you take a low dose of aspirin to reduce your risk of blood clots. Low-dose aspirin may also help reduce burning pain in your feet or hands.


Self care

You can take steps to help yourself feel better if you've been diagnosed with polycythemia vera. Try to:

  • Exercise. Moderate exercise, such as walking, can improve your blood flow. This helps decrease your risk of blood clots. Leg and ankle stretches and exercises also can improve your blood circulation.
  • Avoid tobacco. Using tobacco can cause your blood vessels to narrow, increasing the risk of heart attack or stroke due to blood clots.
  • Avoid low-oxygen environments. Living at high altitudes, skiing or climbing in mountains all reduce the oxygen levels in your blood even further.
  • 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.

  • Avoid extreme temperatures. Poor blood flow increases your risk of injury from hot and cold temperatures. In cold weather, always wear warm clothing, particularly on your hands and feet. In hot weather, protect yourself from the sun and drink plenty of liquids.
  • Watch for sores. Poor circulation can make it difficult for sores to heal, particularly on your hands and feet. Inspect your feet regularly and tell your doctor about any sores.

Preparing for your appointment

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.

What you can do

Make a list of:

  • Your symptoms, including any that seem unrelated to the reason for which you scheduled the appointment, and when they began
  • Key personal information, including other medical conditions and family medical history
  • All medications, vitamins or supplements you take, including doses
  • Questions to ask your doctor

For polycythemia vera, questions to ask your doctor include:

  • What's the most likely cause of my symptoms?
  • What tests do I need?
  • Is this condition temporary, or will I always have it?
  • What treatments are available, and which do you recommend?
  • I have other health conditions. How can I best manage them together?
  • Should I see a specialist?
  • Will I need follow-up visits? If so, how often?
  • Are there brochures or other printed material I can have? What websites do you recommend?

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.

What to expect from your doctor

Your doctor is likely to ask you questions, including:

  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • Does anything worsen your symptoms?

Feb 11, 2022

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  3. Goldman L, et al., eds. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In: Goldman-Cecil Medicine. 26th ed. Elsevier; 2020. https://www.clinicalkey.com. Accessed Dec. 8, 2019.
  4. AskMayoExpert. Polycythemia vera (adult). Mayo Clinic. 2019.
  5. Cuthbert D, et al. Polycythemia vera-associated complications: Pathogenesis, clinical manifestations, and effects on outcomes. Journal of Blood Medicine. 2019; doi:10.2147/JBM.S189922.
  6. Tefferi A, et al. Polycythemia vera treatment algorithm 2018. Blood Cancer Journal. 2018; doi:10.1038/s41408-017-0042-7.
  7. Tefferi A. Prognosis and treatment of polycythemia vera. https://www.uptodate.com/contents/search. Accessed Dec. 7, 2019.
  8. Griesshammer M, et al. Thromboembolic events in polycythemia vera. Annals of Hematology. 2019; doi:10.1007/s00277-019-03625-x.
  9. Spivak JL. Polycythemia vera. Current Treatment Options in Oncology. 2018; doi:10.1007/s11864-018-0529-x.
  10. Van de Ree-Pellikaan C, et al. Treatment strategies for polycythemia vera: Observations in a Dutch "real-world" cohort study. European Journal of Haematology. 2019; doi:10.1111/ejh.13291.
  11. Ferrari A, et al. Clinical outcomes under hydroxyurea treatment in polycythemia vera: A systematic review and meta-analysis. Haematologica. 2019; doi:10.3324/haematol.2019.221234.

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