When you’re diagnosed with polycythemia vera (PV), your doctor will determine whether your PV is low risk or high risk. Risk level refers mainly to your chance of developing a blood clot.

Low-risk and high-risk PV are treated differently. High-risk PV usually involves more intensive treatment, while low-risk PV often needs less intensive treatment.

Having high-risk PV may also bring stress and worry.

“I hate these times of living within the ‘high-risk’ category,” wrote one member of myMPNteam. Another shared, “The sun is shining, the flowers are blooming, I’m feeling good, and I’m trying not to think so much about the risk of a blood clot.”

In this article, we explain the difference between the two risk levels, how they’re typically treated, and which questions to ask your doctor.

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How Polycythemia Vera Risk Level Affects Your Treatment

No matter your risk level, two treatments form the foundation of PV care for most people:

• Phlebotomy — Regular blood draws lower the percentage of red blood cells in your blood and reduce blood thickness.
• Low-dose aspirin — This medication helps keep platelets, the blood cells involved in clotting, from sticking together.

Phlebotomy lowers your iron levels over time, which is part of how it helps control red blood cell production. Do not take iron supplements unless your doctor recommends them. If low iron levels are causing symptoms, ask your doctor how to manage them.

Low-Risk PV Treatment

For many people with low-risk PV, phlebotomy and low-dose aspirin are enough to reduce the risk of blood clots.

Your doctor will monitor your blood counts with regular checkups and adjust your treatment if needed. For example, if aspirin causes side effects or your blood counts become harder to control, your doctor may recommend a different treatment plan.

“The sun is shining, the flowers are blooming, I’m feeling good, and I’m trying not to think so much about the risk of a blood clot.”

— A myMPNteam member

Some people in the low-risk group are advised to start additional treatment for PV.

Your doctor may recommend adding medication for reasons such as:

• Severe or hard-to-control symptoms
• Frequent phlebotomy or difficulty tolerating it
• An enlarged spleen that is growing or causing symptoms
• Rising white blood cell or platelet counts

High-Risk PV Treatment

If you’re in the high-risk group, your hematologist will likely recommend adding cytoreductive medication, which slows how many blood cells your bone marrow makes. Lowering blood cell counts can help reduce the chance of dangerous blood clots.

Common cytoreductive treatment options, usually added to phlebotomy and low-dose aspirin, include:

• Hydroxyurea — A medicine taken by mouth that is often a first choice for cytoreductive treatment
• Ropeginterferon alfa-2b-njft (Besremi) — An injected medicine that lowers blood cell counts and may be used as an alternative to hydroxyurea
• Ruxolitinib (Jakafi) — Often prescribed when hydroxyurea does not work well or causes side effects that are hard to tolerate

Even if you’re taking one of these medications, you may still need occasional phlebotomy.

Rusfertide (Mimrylo) is a new type of drug approved by the U.S. Food and Drug Administration (FDA) to treat people whose PV isn’t well controlled with other therapies. In a clinical trial, rusfertide was shown to reduce the need for phlebotomy.

If you’ve had a blood clot in a vein, your doctor may also prescribe an anticoagulant (blood thinner). The specific medication — and whether you should also take aspirin — depends on your overall health and bleeding risk.

Your treatment plan isn’t based on your risk level alone. Your doctor may also look at your blood counts, symptoms such as itching, and other medical conditions when deciding what treatment to recommend.

Talk to your doctor about your treatment goals and preferences, how side effects affect your quality of life, and whether you’re considering starting a family. These factors are also important as you and your care team decide on your treatment plan together.

Polycythemia Vera Prognosis by Risk Level

Risk level is designed mainly to estimate blood clot risk, but it may also give doctors some information about your overall outlook. In a large study of more than 2,500 people with PV, the estimated 4-year survival rate after study enrollment was 97 percent for people in the low-risk group and 86 percent for those in the high-risk group.

“Picked up my meds today. I’ve come to terms and accept that possible hardship and risk are necessary for future longevity.”

— A myMPNteam member

Because age is one of the factors used to define high-risk PV, these numbers do not show that the risk label itself caused the difference in survival.

Complications from blood clots were the most common known cause of death in the study. That’s why preventing blood clots remains one of the main goals of PV treatment, regardless of your risk level.

Following your treatment plan and keeping regular appointments can help lower your risk of complications.

Does High-Risk PV Mean You’re Going To Die?

No. A high-risk label doesn’t mean death is near or your time is limited.

High risk mainly describes your chance of developing a blood clot compared with someone in the low-risk group. It does not predict exactly how long you will live.

With ongoing treatment and follow-up, many people — including those in the high-risk group — lead active lives for many years after diagnosis.

Does High-Risk PV Mean It’s More Likely To Progress?

PV can sometimes progress to myelofibrosis, another type of MPN, or acute myeloid leukemia. However, the standard low-risk and high-risk labels are designed mainly to estimate blood clot risk, not to predict progression. Your doctor may consider other features when discussing your personal progression risk.

Talk to Your Doctor About Your Polycythemia Vera Risk Level

Understanding your own risk level can help you feel more informed and involved in your PV care. Consider bringing these questions to your next appointment:

• What’s my current risk level, and what factors put me in that category?
• Could my risk level change in the future?
• What would change about my treatment plan if my risk level changed?
• Besides age and clot history, are there other factors affecting my personal risk?
• How often should I have blood tests or checkups based on my risk level?
• Do I need to take an anticoagulant?

Your hematologist can explain the details of your risk level. They can also discuss potential changes to your treatment plan and what to expect over the years as you live with PV.

Living With High-Risk PV

Members of myMPNteam often discuss how they feel about living with high-risk PV and share what helps them cope. Some find it helpful to learn about treatment options, while others try to focus on healthy changes they can make.

“I don’t let myself focus on the what-ifs of the future. Progression will come, or it won’t, and we control what we can. Take our meds, maintain a healthy diet and lifestyle, and go on.”

— A myMPNteam member

“I’ve had TIAs [ministrokes], a heart attack, and blood clots before PV even came up,” said a myMPNteam member. “I’m very high risk. I’ll dive into learning about all options and research.”

“I don’t let myself focus on the what-ifs of the future,” wrote another. “Progression will come, or it won’t, and we control what we can. Take our meds, maintain a healthy diet and lifestyle, and go on.”