Polycythemia vera (PV) is a disorder in which the bone marrow produces too many blood cells, leading to blood clots, bleeding, or an enlarged spleen. PV has a reduced life expectancy due to the possible deadly complications.

Getting treatment can help reduce the risk of certain complications from PV, including blood clots. As a result, a person will likely lead a longer and healthier life with this disease if they receive treatment.

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Photography courtesy of The Armed Forces Institute of Pathology/Wikimedia

According to a 2018 article, the median survival time for people with PV who receive treatment is 14 years after diagnosis. The authors take this survival time from a study in which half of the participants were still alive 14 years after diagnosis.

Younger people tend to live longer with the disease. Research suggests that the median survival time for those under 60 years of age is 24 years following diagnosis.

Multiple factors affect the outlook and life expectancy of people with PV, including:

  • age and overall health
  • presence or absence of certain genetic abnormalities
  • levels of white blood cells
  • development of blood clots, myelofibrosis, or other complications

Blood clots are the most common cause of death in people with PV. When blood clots form in blood vessels, they can block the flow of blood to vital organs. This can lead to life-threatening complications, such as stroke, heart attack, and venous thrombosis.

Treatment for PV can help relieve symptoms and lower the risk of blood clots. In this way, it also reduces a person’s risk of life-threatening complications.

A European study found that the median survival of untreated symptomatic patients with the disease is 6-18 months, whereas, as U.S. research confirmed, people who do get some kind of treatment, may survive a decade or more.

Moreover, people living with PV who do not receive treatment are more likely to develop blood clots. According to the Leukemia & Lymphoma Society, 40–60% of people with untreated PV may develop blood clots within 10 years of diagnosis.

In most cases, healthcare providers prescribe regular blood draws to treat PV. Blood draws reduce the number of blood cells in the body, which may help improve blood flow.

Healthcare providers may also prescribe low-dose aspirin to help prevent the formation of blood clots. Additionally, they may prescribe other medications, such as hydroxyurea (Hydrea) or busulfan (Myleran).

If a person develops MF as a complication of PV, their healthcare provider may prescribe one or more of the following treatments:

  • blood transfusions
  • stem cell transplant
  • medications

In 2021, the U.S. Food and Drug Administration (FDA) approved a new medication Ropeginterferon alfa-2b (Besremi) for treating Polycythemia vera. This drug is now preferred as a first-line treatment due to the fewer side effects associated with it.

It’s also the first medication a doctor can give a patient without considering their treatment history. Other medications a doctor may prescribe include ruxolitinib (Jakafi) or fedratinib (Inrebic).

These treatments may help improve symptoms, increase life expectancy, or both. For example, scientists have found that stem cell transplants may help improve long-term survival in people with MF.

However, this treatment comes with a high risk of life-threatening side effects. It is especially risky for older adults and people with other health conditions. As a result, healthcare providers often avoid prescribing this treatment.

Some studies have found that treatment with JAK inhibitors may also improve survival rates in people with MF. However, when scientists reviewed the available evidence on Jakafi and Inrebic, they found that the quality of evidence on survival rates is limited. More research is necessary to confirm how these medications affect life expectancy, especially on Besremi.

Scientists have not yet developed a cure for PV. However, healthcare providers may recommend drawing blood for testing, medications, or other treatments to help manage symptoms, reduce the risk of complications, and increase life expectancy in people with this disease.

Researchers are also continuing to develop and test potential new treatments for PV, such as the anticancer drug imatinib mesylate (Gleevec) and novel types of JAK inhibitors.

In some cases, a person’s healthcare provider may encourage them to take part in a clinical trial. In this type of study, participants receive an experimental treatment. People interested in learning more about the potential benefits and risks of taking part in a clinical trial can talk with their healthcare provider or the researchers running the study.

Can you live 40 years with polycythemia vera?

With treatment, it is possible to live with polycythemia vera for 35 years or more, as long as the person does not experience a fatal complication such as a stroke or heart attack.

Can you live a full life with polycythemia?

When the condition is managed effectively by a doctor, a person should be able to live a normal life with little interruption.

What is end-stage polycythemia vera?

While rare, the final stage of polycythemia vera can result in chronic leukemia myelofibrosis (MF), which is when scar tissue replaces the bone marrow.

When this happens, the bone marrow can no longer produce enough healthy blood cells. This can sometimes lead to acute myeloid leukemia. The symptoms at this stage include:

  • fatigue and weakness
  • difficulty breathing
  • losing weight
  • night sweats
  • itchiness or bruising
  • pain in the bones or joints

What is the average age of diagnosis for polycythemia vera?

Diagnosis of polycythemia vera happens on average around age 60 and is relatively uncommon below that age, especially if the person has no history of blood clots.

When a person receives a diagnosis of PV, getting treatment is important. Treatment may help minimize symptoms, lower the risk of complications, and improve life expectancy.

A person’s recommended treatment plan for PV will depend on many factors, including their age, overall health, and whether they have developed certain complications.

People with PV who wish to learn more about their treatment options and outlook should talk with their healthcare provider.