Polycythemia vera (PV) is a chronic form of blood cancer. There’s no cure, but that doesn’t mean you shouldn’t get treatment or don’t have options.

With PV, the purpose of treatment is to control abnormal red blood cell replication. This will help reduce the thickness of your blood, allowing you to get more oxygen. The more oxygen you get, the fewer symptoms you’re likely to experience.

Another goal of treatment is to reduce complications and side effects. This includes headaches and itchiness, which are caused by a blockage of blood flow.

When you’re properly treated, you’ll be able to live a productive life with PV for many years. Read about why you shouldn’t delay treating your PV and what options are available to you.

Treatment typically begins by undergoing phlebotomy, a procedure similar to a regular blood donation in which excess blood is removed and discarded from your body.

According to a 2017 study, people with PV tend to undergo the procedure about 8 times a year, those this may vary based on your health, risk factors, and your healthcare professional. The amount of blood withdrawn is around 500 milliliters (ml) in low risk patients and may be higher in some cases.

While undergoing phlebotomy, you may need to take a daily, low dose aspirin. This can help reduce your chance of blood clots. Regular phlebotomy can help reduce your body’s red blood cell volume and help stabilize your blood counts.

Other than aspirin, you have a few options for medications:

Medications that reduce red blood cell count

Some medications work to reduce the number of excessive blood cells in your blood or in your bone marrow. These include:

  • Hydroxyurea: This is a mild form of chemotherapy that limits the number of cells made by the bone marrow. Doctors prescribe it if you’re at higher risk than others for blood clots. Some common side effects include allergic reactions, low blood counts, infection, and others.
  • Ruxolitinib (Jakafi): This is a Janus Kinase (JAK) inhibitor for those with advanced PV who are unable to tolerate hydroxyurea or when hydroxyurea does not work well. It stops the action of the JAK2 mutation, which occurs in the majority of those with PV.
  • Interferon alfa: This is often prescribed to younger patients or pregnant people. This drug is injected with a needle, typically three times a week. It will prompt your body to lower your blood cell counts. One of the main disadvantages of this drug is its expensive price tag.
  • Busulfan (Myleran): This cell cycle non-specific alkylating antineoplastic agent that disrupts the production of blood cell DNA in the bone marrow, causing excessive blood cells to die.

Medications that reduce itching

PV can often cause pruritus or itchy skin. The following drugs may provide relief:

  • Antihistamines: These are allergy medications and include diphenhydramine (Benadryl) or doxepin.
  • Psoralen: This is administered as part of as part of light therapy (phototherapy). Psoralen increases the amount of ultraviolet (UV) light absorbed by your skin, which can help reduce symptoms of various skin conditions.
  • Anticonvulsants: Drugs like gabapentin or pregabalin stop neurotransmitters in the central nervous system that would otherwise send signals leading to itchiness.

One of the last or final treatment options is a bone marrow transplant. In fact, this is the only treatment with the potential to cure the disease

You may consider this option when your PV is advanced, and all other treatments have been ineffective. You may also think about having a transplant if you experience serious bone marrow scarring and are no longer producing healthy, functioning blood cells.

After a successful bone marrow transplant, you will no longer have symptoms of PV. This is because the stem cells, where the disease originates, will be replaced.

However, complications from a bone marrow transplant can be serious. This includes rejection of the stem cells by your body and damage to your organs. Make sure to discuss having a bone marrow transplant thoroughly with your hematologist beforehand.

Because there’s no complete cure for PV, advances in research and treatments are ongoing.

The discovery of the JAK2 gene and its mutation was a huge step forward in the understanding of PV. A search for the cause of this mutation continues, and once it’s found, you may see even more advances in treatment options.

Clinical trials are another way in which new treatments are being evaluated and tested. Currently, a variety of other medications are being explored as potential treatments.

Your hematologist may also speak with you about clinical trials if you’re at higher risk for blood clots.

There are a few lifestyle changes you can make that can help support your treatment. These include:

  • drinking plenty of water
  • quitting smoking
  • exercising
  • getting enough sleep
  • eating a balanced diet

In addition, speak to your doctor about vaccinations you may need to get to support your immune system. If you need support with your mental health, resources are also available.

Can you live a normal life with polycythemia vera?

With proper treatment, most people can live for at least 10 years post-diagnosis. Younger people, and those who are low risk, can live much longer.

What is the drug of choice for polycythemia vera?

Typically, the first-line treatment for PV is phlebotomy in combination with daily aspirin, followed by other medications.

Should I worry if I have polycythemia?

Without proper diagnosis and treatment, PV can be fatal. It can cause blood clots, which can lead to a heart attack, stroke, or pulmonary embolism. Additionally, your liver and spleen may enlarge, causing more complications. However, with appropriate treatment, you can greatly reduce the chance of these serious complications.

Your hematologist, a specialist in blood diseases, will be the doctor in charge of your treatment.

They will discuss your specific treatment options, which will take into account the stage of your disease, your age, your risk of blood clotting, and your level of tolerance.

No matter what course of treatment you and your hematologist decide is best, know that PV can be controlled and managed effectively.