Polycythemia vera symptoms

Polycythemia vera (PV) is a chronic blood cancer that causes your body to produce too many red blood cells, with many people also often having elevations in white blood cells and platelets, which help blood to clot.

As these extra blood cells build up, your blood can become thicker than normal and flow less easily through your blood vessels. This can lead to a wide range of symptoms and increase your risk of serious complications.

Understanding PV symptoms can help you recognize possible signs of the disease, know when to talk with your healthcare team, and identify symptoms that require prompt medical attention.

What are the main symptoms of PV?

PV symptoms can range from mild to life-threatening. The disease develops slowly and often silently, and many people have few or no symptoms when they are first diagnosed. However, as the disease progresses, you may experience a range of symptoms that affect your daily life.

Symptoms of PV may include:

• itchy skin, especially after warm baths or showers, known as aquagenic pruritus
• redness and burning or tingling in your limbs
• fatigue
• headaches
• neurological symptoms such as difficulty concentrating, dizziness, or changes in your vision
• high blood pressure
• enlargement of your spleen, which can cause abdominal pain
• shortness of breath and trouble breathing while lying down
• abnormal bleeding or hemorrhage

Because blood is too thick and viscous, the risk of having blood clots, known as thrombosis, is increased in people with PV. Symptoms of blood clots vary depending on where the clot is, but some can be serious. If you experience slurred speech, weakness on one side of the body, sudden chest pain, or any other symptoms that seem sudden and abnormal, call 911 or go to the emergency department of your local hospital or care center.

Early symptoms of PV

Not everyone with PV develops symptoms early in the disease. If you do experience early symptoms of PV, they are often mild and easy to overlook.

Early symptoms may include:

• fatigue
• headaches
• difficulty concentrating
• dizziness or lightheadedness
• insomnia
• redness, warmth, and pain in your extremities, called erythromelalgia
• aquagenic pruritus
• abdominal pain

Itching, redness, and burning sensations

PV itching is a common and bothersome symptom of the disease. Many people with PV develop aquagenic pruritus, a type of itching that begins during or after contact with water. Most people report that warm water causes more intense itching, although some say the temperature of the water does not seem to matter, and a few have worse symptoms when in contact with cold water.

About 40% of people with PV experience aquagenic pruritus, and it is often one of the earliest signs of the condition. Researchers do not fully understand what causes PV itching. However, the release of histamine, a chemical involved in allergic reactions, is believed to contribute to this symptoms.

Beyond itching, aquagenic pruritus can also be experienced as tickling, stinging, and burning sensations. However, because PV makes your blood thicker than normal, it can also reduce blood flow to your hands and feet. This may also cause redness, tingling, burning, or pain in your extremities, specifically your fingers and toes. 

Symptoms caused by an enlarged spleen

Your spleen helps filter your blood, remove old or damaged blood cells, and support your immune system. In people with PV, it may also begin producing blood cells when your bone marrow, the soft tissues inside bones where most blood cells are made, cannot keep up with your body’s needs. As a result, your spleen can become enlarged, a condition called splenomegaly.

If your spleen becomes enlarged, you may experience symptoms such as:

• pain in the upper left region of your abdomen
• feeling full after eating small amounts of food

Your healthcare provider may be able to feel an enlarged spleen during a physical exam. In some cases, imaging tests also may be used to confirm the diagnosis.

Blood clot warning signs

One of the most serious complications of PV is a greater risk of blood clots. PV may increase your risk of clots forming in arteries and veins. If the blood vessels that supply your brain are affected, it can lead to a stroke.

In some people, a blood clot or stroke may sometimes be the first sign of PV.

The symptoms of thrombosis depend on where the clot forms, but warning signs may include the following:

• sudden pain, swelling, or warmth in one leg or arm
• sudden abdominal pain or nausea
• sudden chest pain and shortness of breath
• very fast breathing or heart rate
• severe headache
• sudden weakness affecting one side of the body
• difficulty speaking or walking

Blood clots and strokes are medical emergencies that can be life-threatening. If you develop symptoms that could suggest either condition, seek emergency medical care right away.

Bleeding and bruising symptoms

Although blood clots are generally more common, PV can also increase your risk of abnormal bleeding. Bleeding may affect many parts of your body, and it may manifest as nosebleeds, bleeding gums, bruising easily, or having heavy periods.

You also may be more likely to experience excessive bleeding, bruising, or blood clotting after surgery, dental work, or other medical procedures.

Do symptoms differ between men and women?

Although many PV symptoms are similar in men and women, research suggests there are some differences in how the disease may develop and present. PV symptoms in women tend to be milder in some ways, but women may experience certain symptoms or complications more commonly than men.

For example, women with PV tend to have:

• a lower percentage of red blood cells in the blood (hematocrit)
• smaller spleens
• a lower risk of arterial thrombosis, which occurs in the arteries

At the same time, women may be more likely to experience:

• PV headaches
• gastrointestinal symptoms
• venous thrombosis, including blood clots in unusual locations, such as the veins that drain blood from the digestive system to the liver

PV symptoms in men may include a higher risk of arterial thrombosis and a tendency for the disease to progress more quickly.

Although these differences have been reported in research studies, every person experiences PV differently. Your symptoms and risk of complications depend on many factors, including your overall health and how well your condition is controlled.

Symptoms that may suggest disease progression

PV is a chronic disease that can change over time. In some people, it may progress to more advanced blood cancers, including myelofibrosis, a condition in which scar tissue replaces the bone marrow, or acute myeloid leukemia.

Symptoms that may suggest PV is progressing include:

• more frequent or severe PV symptoms, such as itching episodes or thrombosis
• increased fatigue
• night sweats
• unintended weight loss
• frequent infections
• fever
• new or increasing symptoms of an enlarged spleen

These symptoms do not necessarily mean your disease has progressed, but they are important to discuss with your healthcare provider.

If you notice any new symptoms or changes in your existing PV symptoms, let your healthcare team know. Identifying changes early can help your care team determine whether your treatment plan needs to be adjusted and monitor for possible complications or disease progression.

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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.