For decades, phlebotomy, or removing blood from the body, has been a mainstay of treatment for polycythemia vera (PV). It helps lower the number of red blood cells and reduce the risk of dangerous blood clots.

Today, there are several other treatments that can help control PV, manage symptoms, and lower the risk of serious complications. Doctors recommend treatments based largely on whether someone has a low-risk or high-risk PV.

In this article, we’ll cover treatment options for both low-risk and high-risk groups, along with promising therapies currently under development. Knowing what’s available can help you have better conversations about treatment with your blood cancer specialist.

🗳️ Have you discussed PV treatments other than phlebotomy and low-dose aspirin with your blood cancer specialist?

No, I haven’t discussed other PV treatments.

Yes, but I’m still only using phlebotomy and low-dose aspirin to treat PV for now.

Yes, and my treatment plan includes medications beyond low-dose aspirin.

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Treatment Options for Low-Risk Polycythemia Vera

If you have low-risk PV, treatment usually focuses on preventing blood clots and keeping your blood counts under control.

Low-Dose Aspirin

Although low-dose aspirin isn’t specifically approved by the U.S. Food and Drug Administration (FDA) to treat PV, it’s widely recommended in treatment guidelines and is considered a standard part of care for most people with the condition.

Low-dose aspirin helps make platelets less “sticky,” reducing the chance that blood clots will form. Unless there’s a reason you shouldn’t take it, such as certain bleeding disorders or an aspirin allergy, most people with PV are advised to take a daily low-dose aspirin.

It’s important to take low-dose aspirin only as directed by your healthcare provider.

Interferon Therapy

Ropeginterferon alfa-2b-njft (Besremi) is FDA-approved to treat adults with polycythemia vera. Other forms of pegylated interferon, such as peginterferon alfa-2a (Pegasys), may also be prescribed off-label, which means they’re approved for other conditions but are sometimes prescribed by specialists to treat PV.

Interferons help regulate the immune system and reduce production of blood cells in the bone marrow. Ropeginterferon alfa-2b-njft can help control blood counts and may reduce the need for repeated phlebotomies in some people.

Interferon therapy is an option for adults with both low-risk and high-risk PV, depending on their treatment goals and overall health.

Symptom-Directed Treatments

Some medications don’t treat the underlying disease but can help relieve symptoms that affect daily life. Depending on your symptoms, your doctor may recommend treatments for severe itching, gout, or iron deficiency.

Managing symptoms is an important part of living with PV. Even when blood counts are well controlled, treating bothersome symptoms can improve comfort and quality of life.

Treatment Options for High-Risk Polycythemia Vera

People with high-risk PV are generally age 60 or older, have had a previous blood clot, or have other features that increase their risk of complications.

Along with phlebotomy and aspirin when appropriate, many people in this group receive medications that reduce blood cell production. These medications are known as cytoreductive therapies, meaning they lower the number of blood cells the bone marrow produces.

Hydroxyurea

Although hydroxyurea isn’t FDA-approved specifically for PV, it’s recommended as a first-line treatment for many people with high-risk disease.

Hydroxyurea slows the bone marrow’s production of red blood cells and other blood cells. It’s one of the most commonly prescribed medications for people with high-risk PV because it helps control blood counts and lowers the risk of blood clots.

People taking hydroxyurea need regular blood tests so their healthcare team can monitor blood counts and adjust the dose if needed. If hydroxyurea doesn’t control PV well enough or causes side effects that are difficult to manage, your doctor may recommend another treatment.

Interferon Therapy

Some doctors prescribe ropeginterferon alfa-2b-njft and peginterferon alfa-2a to treat high-risk PV.

Some specialists recommend interferon therapy instead of hydroxyurea for certain people, including younger adults or those who don’t tolerate hydroxyurea well.

Ruxolitinib

Ruxolitinib (Jakafi) is FDA-approved for adults with PV whose disease hasn’t responded well to hydroxyurea or those who can’t tolerate it.

Ruxolitinib is a targeted therapy called a JAK inhibitor. It blocks an overactive signaling pathway that contributes to the overproduction of blood cells in PV.

Ruxolitinib is typically prescribed after hydroxyurea if that medication isn’t working well enough or causes unacceptable side effects.

In addition to helping control blood counts, ruxolitinib may improve symptoms such as itching, night sweats, and an enlarged spleen.

Rusfertide

Rusfertide (Mimrylo) was recently approved to treat people whose PV isn’t well-controlled with other therapies. Rusfertide is a laboratory-made version of hepcidin, a natural hormone that helps regulate how the body uses and stores iron.

Rusfertide reduces red blood cell production by limiting the iron available to make new red blood cells. In clinical trials, rusfertide decreased the need for phlebotomy.

What’s Next in PV Treatment?

Researchers are developing new therapies that could reduce the need for frequent phlebotomies while helping control blood counts and improve quality of life. Although these treatments aren’t yet widely available, several are showing promise in clinical trials.

Givinostat

Givinostat is another investigational therapy being studied for PV. It works differently from currently available treatments by targeting proteins involved in abnormal blood cell growth. Early studies have been encouraging, but more research is needed before it becomes available.

Other Investigational Therapies

Researchers are also studying additional targeted therapies and new combinations of existing medications to better control blood counts, reduce symptoms, and potentially slow disease progression.

While it’s too early to know which approaches will become standard treatments, the growing number of therapies in development offers hope for more personalized care in the future.

If you’re interested in investigational treatments, ask your PV specialist whether a clinical trial might be right for you.

More Options, More Conversations

Phlebotomy remains an important part of treating PV, but it’s no longer the only option. Depending on your risk level, symptoms, and treatment history, your care plan may also include medications that lower blood count, targeted therapies, or newer treatments designed to reduce the need for repeated blood draws.

As researchers continue to develop new therapies, the outlook for people living with PV continues to evolve. Talking with your hematologist or PV specialist about all of your treatment options can help ensure your care plan matches your current needs and long-term goals.