Polycythemia vera treatment
Fact-checked by Polycythemia vera (PV) is a rare, slow-growing blood cancer that causes your body to produce too many red blood cells. It can also increase the levels of your white blood cells and platelets, the blood cell fragments that help your blood clot. Although PV cannot be cured, treatment can help manage your symptoms, lower your risk of blood clots, and reduce the likelihood of complications.
The right PV treatment, along with regular follow-up, can help you manage your condition and maintain your quality of life.
PV treatment options
Current treatment strategies for PV focus on lowering your risk of blood clots, managing your symptoms, and preventing long-term complications.
Your treatment plan depends on factors such as your age, risk of blood clots, symptoms, blood cell counts, and the effectiveness of previous treatments. Many people receive a combination of treatments rather than a single therapy.
The foundation of treatment for most patients includes:
- Phlebotomy: During phlebotomy, a healthcare provider draws a specific amount of blood, similar to a blood donation, to lower your hematocrit, or the percentage of red blood cells in your blood. Removing excess blood makes your blood less thick, helping lower your risk of blood clots. This procedure may be repeated every few weeks or months, depending on your needs.
- Low-dose aspirin: Low-dose aspirin helps prevent platelets from sticking together, making blood clots less likely to form. Your doctor may recommend taking low-dose aspirin every day if the medication is safe for you.
When phlebotomy and aspirin are insufficient to control your blood counts or symptoms, or if you have a higher risk of developing blood clots, your doctor may recommend additional treatments.
Cytoreductive therapy
Cytoreductive therapy uses medications to reduce abnormally high blood cell counts. These therapies work to:
- lower blood cell production
- reduce hematocrit levels
- lower the risk of blood clots
- manage PV symptoms
Hydroxyurea
Hydroxyurea is an oral medication taken every day. It helps slow the production of new blood cells in your bone marrow, the spongy tissue inside bones where most blood cells are formed. Hydroxyurea is one of the most commonly used cytoreductive therapies in PV and is effective for many people.
Some people eventually stop responding to hydroxyurea or develop side effects that make it difficult to continue treatment. Because hydroxyurea is a form of chemotherapy, it should not be used during pregnancy, so talk with your doctor about other treatment options if you are pregnant or planning to become pregnant.
Interferon
Interferon is an injectable medication that may be given weekly or every other week. Two forms of interferon are available to treat PV: pegylated interferon alpha-2a, which is sold as Pegasys and used off-label for PV, and Besremi (ropeginterferon alfa-2b-njft).
Researchers do not fully understand exactly how interferon works, but it helps reduce the production of excess blood cells and has been shown to be as effective as hydroxyurea for many people.
Interferon is generally considered the preferred cytoreductive therapy during pregnancy when treatment is needed, although it may cause mental health side effects in some people.
Jakafi
Jakafi (ruxolitinib) is another cytoreductive treatment option for PV offered as a second-line treatment for people who cannot tolerate or do not respond well enough to hydroxyurea. It blocks JAK proteins, which help control blood cell production. Mutations in the JAK2 gene cause nearly all PV cases.
Jakafi is an oral medication taken twice daily. Clinical trials suggest it can help control hematocrit levels, reduce spleen size, and lower the need for phlebotomy.
Although generally well tolerated, Jakafi can increase your risk of infections and lower certain blood cell counts.
Busulfan
Busulfan is an oral chemotherapy medication that suppresses bone marrow activity and reduces blood cell production. Doctors usually reserve this option for people who cannot tolerate or no longer respond to hydroxyurea, interferon, or other treatments.
Because busulfan can cause prolonged suppression of blood cell production and other serious side effects, it is usually used less often than other cytoreductive therapies.
How doctors choose a treatment plan
To recommend the treatment plan that is right for you, your healthcare team will consider several factors. One of the most important is your risk of developing a blood clot.
Your healthcare team typically evaluates the following when assessing your risk:
- Age: People younger than 60 are generally considered at lower risk, while those 60 and older are generally considered at higher risk.
- History of blood clots: If you have had a blood clot before, you face a higher overall risk.
People with high-risk PV are often treated with cytoreductive therapy in addition to phlebotomy and low-dose aspirin. People with low-risk PV may need only phlebotomy and low-dose aspirin.
Your healthcare team will also consider your overall health, symptoms, treatment goals, and personal preferences when recommending a treatment plan.
Monitoring during treatment
Regular follow-up appointments are an important part of managing PV. Your healthcare team will likely recommend routine blood tests and regular checkups to monitor your blood cell counts, evaluate how well your treatment is working, and watch for signs that your condition is changing.
These visits also allow your healthcare team to monitor for treatment side effects and adjust your treatment plan if needed. Keeping up with regular appointments can help ensure your treatment continues to meet your needs over time.
Treatments for PV pruritus
Many people with PV experience itchy skin, or pruritus, especially after a warm bath or shower. This symptom, known as aquagenic pruritus, can significantly affect your quality of life. Although standard PV treatments improve itching for some people, they do not work for everyone.
Depending on your symptoms, your doctor may recommend one or more of the following treatments or lifestyle modifications:
- Taking less frequent showers
- Avoiding heated sources of water and opting for cool showers
- Keeping your skin well-moisturized
- Taking medications such as selective serotonin reuptake inhibitors, a type of antidepressant, or antihistamines
- Trying a form of light therapy called narrowband ultraviolet B phototherapy
If itching continues to interfere with your daily activities or sleep, talk with your doctor about additional treatment options.
Questions to ask about PV treatment options
As you discuss your treatment options with your healthcare team, you may want to ask questions such as:
- What treatment options do you recommend for me?
- What side effects might my treatment cause, and which warning signs should I watch for?
- What hematocrit level should I aim for?
- How often will I need follow-up appointments or blood tests?
- Do I need to take any special precautions while receiving treatment?
- Are there any medications, supplements, or over-the-counter products I should avoid?
Managing PV is an ongoing process, and your treatment plan may change over time. Regular follow-up appointments and open communication with your healthcare team can help ensure your treatment continues to meet your needs and reduce your risk of complications.
Rare Cancer News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.
