17742 Polycythemia Vera

Overview

Polycythemia vera (poly-sigh-THEE-me-ah VAY-rah) is a rare blood condition in which your bone marrow makes too many red blood cells. These extra cells make your blood thicker than normal, so it does not flow as easily. This can increase the risk of blood clots. It is also sometimes called PV.

Polycythemia vera is rare. It belongs to a group of conditions called myeloproliferative neoplasms, in which the bone marrow makes too many cells.

It usually affects adults over the age of 60, and it is slightly more common in men. It can occur at any age, but it is very unusual in children and young adults.

Symptoms

Common symptoms

• Feeling very tired or weak
• Headaches or dizziness
• Blurred vision or seeing spots
• Itchy skin, especially after a warm shower or bath
• Red or flushed face and palms
• Ringing in the ears
• Feeling full or discomfort in the upper left area of the belly due to an enlarged spleen

Symptoms in children

• Polycythemia vera is very rare in children, and when it does happen, the symptoms are similar to those in adults, such as tiredness, headaches, and flushing of the skin.

Symptoms in older adults

• Older adults may notice tiredness, confusion, shortness of breath on activity, or less obvious signs of a blood clot. These symptoms can sometimes be mistaken for normal ageing, so it is important to have any new symptoms checked.

Causes

Main causes

• Polycythemia vera happens when a bone marrow stem cell develops a genetic change (mutation) that makes it produce too many red blood cells, and sometimes too many white blood cells and platelets.
• This genetic change is usually acquired during life, not passed down from parents.
• In most cases, the exact reason the genetic change happens is unknown.

Risk factors

• Age over 60
• Being male
• Long-term exposure to certain industrial chemicals or radiation may be linked, but this is not well understood and is less common

When to see a doctor

See a doctor urgently if:

• If you have chest pain, trouble breathing, weakness on one side, sudden vision loss, or trouble speaking, call your local emergency number immediately.
• If you have swelling and pain in one leg or arm, or bleeding that will not stop, seek urgent medical care the same day.

Book a routine appointment if:

• Make a routine appointment with your GP if you have persistent tiredness, headaches, itchiness after a shower, dizzy spells, or an unusually red complexion. A simple blood test can check your blood cell counts.

Diagnosis

A doctor will ask about your symptoms, examine you, and order a blood test called a full blood count. If the test shows a high red blood cell level, your doctor may refer you to a specialist for more tests.

Tests that may be done

• Full blood count with a blood film
• JAK2 mutation blood test, which looks for the genetic change linked to polycythemia vera
• Erythropoietin level test
• Oxygen saturation measurement
• Ultrasound scan of the abdomen to check for an enlarged spleen
• Bone marrow biopsy, if needed

What to expect at your appointment

You will usually be referred to a haematologist, a doctor who specialises in blood conditions. Testing can take a few weeks. The specialist will use your results to decide whether polycythemia vera or another condition is causing your symptoms.

Treatment

Treatment for polycythemia vera aims to lower your blood counts, reduce the thickness of your blood, and prevent blood clots. The plan depends on your age, symptoms, blood counts, and overall risk. You will need regular monitoring, usually for life.

Self-care at home

• Drink plenty of fluids to avoid dehydration
• Stay active and avoid sitting still for long periods, especially on long journeys
• Do not smoke, because smoking increases the risk of blood clots
• Attend all your blood tests and follow-up appointments
• Before taking any new medicine, supplement, or herbal product, ask your doctor

Medical treatments

The main treatment is a procedure called venesection, which means removing a small amount of blood from a vein through a needle. This safely lowers the number of red blood cells. A specialist may also use medicines that slow down how many blood cells your bone marrow makes, and sometimes prescribe a medicine to reduce the risk of blood clots. The exact medicines are individualised, and your specialist will explain the benefits and risks before starting anything.

When is surgery considered?

Surgery is not used to treat polycythemia vera itself. In rare cases, if an enlarged spleen is causing severe pain or other problems, a specialist may discuss surgery to remove the spleen, but this is uncommon.

Living with this condition

You will see your specialist team regularly for blood tests and check-ups. Many people with polycythemia vera continue to work, travel, and enjoy their usual activities. It is important to tell your doctor about any new symptoms, especially signs of a blood clot, and to take any prescribed medicines exactly as directed.

Lifestyle tips

• Keep your blood pressure and cholesterol under control with regular check-ups
• Maintain a healthy weight
• Avoid dehydration, especially in hot weather or during exercise
• If you are travelling on a long flight or car journey, get up and move every hour or so

Diet and exercise

There is no special diet that cures polycythemia vera, but a heart-healthy diet can help. Eat plenty of vegetables, fruits, whole grains, lean protein, and healthy fats. Aim for gentle regular exercise such as walking, swimming, or cycling. Talk to your doctor before starting a vigorous exercise programme, particularly if you have had a blood clot.

Mental health and emotional wellbeing

Living with a chronic blood condition can cause worry, stress, or low mood. These feelings are very normal. If they are affecting your daily life, speak to your GP or your care team. If you feel in crisis or have thoughts of harming yourself, contact a local mental health crisis service or your local emergency number immediately.

Prevention

Polycythemia vera cannot be prevented, because it is caused by an acquired genetic change rather than by lifestyle. However, there are steps you can take to reduce the risk of complications, especially blood clots by following your treatment plan and staying active.

Vaccines

Keep your routine vaccinations up to date. Ask your doctor whether you need any additional vaccines, especially before starting treatment.

Screening programmes

There is no routine screening test for polycythemia vera in the general population. If you have a family history of blood conditions and are worried, talk to your doctor about whether regular blood tests might be useful for you.

Complications

If left untreated

• Blood clots, including deep vein thrombosis, pulmonary embolism, heart attack, or stroke
• Abnormal bleeding, such as easy bruising, nosebleeds, or bleeding in the stomach
• Enlarged spleen, which can cause pain and a feeling of fullness
• High blood pressure and gout
• In rare cases, over many years, the bone marrow may become scarred (myelofibrosis) or develop into a type of blood cancer

Long-term outlook

With regular treatment and monitoring, the outlook for people with polycythemia vera is generally good. The most serious risk is blood clots, and treatment lowers that risk dramatically. Many people live full, active lives for many years after diagnosis.

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