Polycythemia vera (PV) is a rare disorder that affects blood cell and platelet production. The early stages of polycythemia vera often cause nonspecific symptoms, but advanced stages can lead to myelofibrosis and other severe complications.

PV often causes mild or no noticeable symptoms at first. They tend to become more apparent over time.

This article describes the different stages of PV and how healthcare professionals may treat this disease.

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Symptoms of PV tend to develop gradually over years.

In the early stages, the condition may cause no apparent symptoms. A healthcare professional may diagnose it following a routine blood test before any symptoms develop.

As time passes, more noticeable symptoms may occur. The disease may also cause complications, such as an enlarged spleen, chest pain, a heart attack, or a stroke.

PV may eventually lead to myelofibrosis (MF). This happens when scar tissue replaces the bone marrow. Experts sometimes call this the “spent phase” of PV.

A person may not have noticeable symptoms in the early stages of PV. Any symptoms that appear are often nonspecific.

For example, early symptoms may include:

  • fatigue
  • weakness
  • dizziness
  • headache
  • unusual sweating, particularly at night
  • itchy skin, which may get worse after a warm bath or shower

Less common symptoms include:

  • red skin, especially on the face
  • frequent nosebleeds
  • bleeding gums
  • easy bruising
  • ringing ears
  • blurred vision
  • a burning sensation on the skin, especially on the hands and feet
  • heavy bleeding from minor cuts

Also, people with PV have an increased risk of blood clots, which may develop even in the early stages. They can cause potentially life threatening complications, such as a stroke or heart attack.

PV may also cause other complications, including an enlarged spleen. The spleen filters worn-out blood cells from the body. When it becomes enlarged, it can cause a feeling of bloating or fullness in the belly.

This condition can lead to other serious complications, including more frequent infections, anemia, and increased bleeding.

Over time, some people with PV may develop MF. This occurs if scar tissue replaces bone marrow. When a person has MF, the bone marrow cannot produce enough healthy blood cells.

Some symptoms of MF include:

  • fatigue
  • weakness
  • shortness of breath
  • unexplained weight loss
  • easily bruising or bleeding
  • fever
  • unusual sweating, particularly at night
  • pain or a feeling of fullness below the ribs on the left side

People with MF may also develop an enlarged spleen or liver. This may cause discomfort and bloating or a feeling of fullness in the belly. It can also lead to other, more serious complications, including more frequent infections, a greater risk of bleeding, and anemia.

In rare cases, MF develops into acute myeloid leukemia.

Often, PV results from a change in the JAK2gene. This gene instructs the body to make a protein that helps produce blood cells.

PV causes the blood to become thicker than usual. The blood clots more easily and may not flow through the blood vessels properly. As a result, it may carry less oxygen to the body’s tissues and organs.

Over time, these changes can cause noticeable symptoms and potentially life threatening complications.

PV advances more quickly and severely in some people than in others. Not all people with PV develop MF.

A person’s outlook depends on many factors, such as:

  • their age and overall health
  • whether they have certain genetic mutations
  • whether they have high levels of white blood cells
  • the treatment they receive

Experts have not yet found a cure for PV. However, treatments for the disease may help relieve symptoms and reduce the risk of certain complications.

Treating PV

A healthcare professional may recommend a variety of treatments for PV, such as:

  • regular blood draws
  • a low dose of aspirin to reduce the risk of blood clots
  • medication to limit the production of blood cells, such as:
    • hydroxyurea (Hydrea)
    • interferon-alpha (Multiferon)
    • ruxolitinib (Jakafi)

A person may also need other treatments to relieve certain symptoms or treat complications of PV.

Treating MF

If a person develops MF, the doctor may recommend regular blood transfusions, iron supplementation, and folate supplementation.

They may also prescribe a medication called a Janus kinase inhibitor, such as ruxolitinib (Jakafi) or fedratinib (Inrebic).

In rare cases, they may recommend a stem cell transplant. However, this treatment carries a high risk of life threatening side effects.

If a person’s spleen becomes very enlarged, the doctor may recommend surgery to remove it.

If acute myeloid leukemia develops, the doctor may prescribe chemotherapy, targeted therapy, or other treatments.

PV raises a person’s risk of potentially life threatening complications, such as a heart attack, stroke, or acute myeloid leukemia.

Early treatment is important for relieving symptoms and reducing the risk of complications. Getting treatment may help improve a person’s quality of life and increase life expectancy.

After receiving a diagnosis of PV, a person should attend regular checkups with their healthcare professional, who may order periodic blood tests to learn how the disease is progressing. If needed, the doctor will recommend changes to the treatment plan.