Polycythaemia vera (PV)

Having a high number of red blood cells does not always cause symptoms. Some people are diagnosed with PV after they have a blood test for another reason.

Possible symptoms of PV include:

• severe, migraine-type headaches
• changes in vision
• feeling very tired (fatigue)
• feeling dizzy
• itchy skin (often after a warm bath or shower)
• sweating more than usual
• burning pain, change in feeling, or redness in the hands or feet
• cold or blue fingers or toes
• redness of the face
• bone or joint pain
• unexplained weight loss
• high temperature (fevers) and drenching night sweats requiring a change of clothes or bed covers
• discomfort in the tummy area (abdomen)
• feeling full soon after beginning to eat.

Some people also develop symptoms because of complications of PV.

Enlarged spleen

The spleen sits on the left side of the tummy, just under the ribs. It is usually about the size of a fist.

The spleen:

• helps fight infection
• stores and breaks down blood cells.

PV can cause the spleen to grow bigger than normal. This is called an enlarged spleen. Sometimes, this causes symptoms. These include discomfort on the left side of the tummy and feeling full quickly when eating.

Having an enlarged spleen can cause problems. It may:

• keep blood cells in the spleen instead of releasing them into the blood
• destroy blood cells.

Both these changes can reduce the number of blood cells in the blood.

Blood clots

Some people with PV have a higher risk of developing a blood clot. Symptoms of a blood clot include:

• throbbing pain, redness or swelling in a leg or arm
• suddenly feeling breathless or coughing
• sharp chest pain, which may be worse when you cough or take a deep breath.

If you have any of these symptoms, contact the hospital straight away. If you cannot speak to your doctor, call the NHS urgent advice number on 111.

A blood clot is serious, but it can be treated with drugs that thin the blood (anti-coagulants). Your doctor or nurse can give you more information.

You may be given drugs to help prevent a clot.

Blood clots can also cause heart attacks and strokes. If you think you may be having a heart attack or stroke, call 999 for an ambulance or go straight to A&E (emergency department).

Gout

When the body tries to break down (get rid of) more blood cells than usual, it can cause a painful condition called gout. Gout affects the joints. It causes pain, heat, redness and swelling. It usually only affects the big toe joint. But it can affect other joints.

Gout is caused by uric acid building up in the blood. Uric acid is produced when lots of cells are broken down. The uric acid forms crystals that get into the joint and cause the symptoms.

Usually, the kidneys can clear uric acid from the blood and pass it out of the body in the pee (urine). But sometimes, they cannot cope with large amounts.

Your doctor can check the level of uric acid in the blood. Depending on the result, they may give you a tablet called allopurinol to help prevent gout.

Bleeding or bruising

Sometimes, PV can cause abnormal bleeding or bruising. Any bleeding might be heavier than normal and take longer to stop.

Symptoms may include:

• nosebleeds
• bleeding gums
• heavy periods
• blood in your pee or stools (poo)
• very dark poo blood in your vomit (sick) – this can be a bright red liquid or look like brown or black coffee granules
• tiny red or purple spots on your skin that may look like a rash.

Tell your doctor if you notice any signs of abnormal bleeding or bruising.

If you think you see blood in your sick or poo, or if you have dark poo, or if any bleeding is heavy or will not stop, contact a doctor urgently. If you cannot speak to a doctor, call 999 for an ambulance or go straight to A&E.

Myelofibrosis (MF)

A very small number of people with PV may develop a more serious condition called myelofibrosis (MF). MF causes scarring of the bone marrow.

Acute myeloid leukaemia (AML)

Rarely, PV can cause a cancer of the blood cells called acute myeloid leukaemia (AML). Not everyone with PV has the same risk of developing AML.

For most people, PV remains stable for many years and does not affect how long they live for. Your doctor can tell you more about this.

Before treatment, a team of health professionals work together to plan the treatment they feel is best for you. This team is called a multi-disciplinary team (MDT).

The MDT look at the risk of you developing problems from PV. They also look at your test results and general health. They will describe the PV as low, intermediate (medium) or high risk based on:

• your age
• your symptoms
• your medical conditions
• your number of blood cells
• whether you have genetic changes
• the thickness of your blood (haematocrit level)
• whether you have had blood clots.

Treatment for PV is usually given to prevent problems such as blood clots. This is called supportive treatment. Treatment can also be given to control PV by reducing the number of red blood cells (cytoreductive therapy). You will have regular blood tests to check for changes in your condition.

You and your doctors will talk about your treatment options and the possible risks and benefits before you agree (consent) to have treatment.

You may be offered treatment as part of a clinical trial.

If you have PV, you have a higher risk of blood clots. Blood clots can cause heart attacks and strokes. It is important to reduce your risk as much as possible.

It is important that other conditions that can also increase your risk of heart attacks and strokes are managed. This includes conditions such as:

• diabetes
• high blood pressure
• high cholesterol.

Lifestyle changes

Your doctors and nurses will give you advice about any lifestyle changes you can make to help reduce your risk. They usually advise you to:

• give up smoking, if you smoke
• drink plenty of fluids
• eat healthily and be physically active
• keep to a healthy weight
• reduce or manage stress.

Aspirin

Most people with PV are treated with aspirin tablets. Aspirin affects the way platelets stick together and helps prevent blood clots. Drugs that prevent clots are called anti-platelet drugs or blood thinners. Aspirin does not affect the number of platelets in the blood.

One of the side effects of aspirin is an increased risk of bleeding and ulcers in the stomach.

If you are taking aspirin, ask your doctor for advice about painkillers. Certain types of painkillers, such as ibuprofen, may not be suitable for you while you are taking aspirin.

If you cannot take aspirin because of medical conditions such as a stomach ulcer, you may be given another anti-platelet drug instead.

If you have PV, you need regular check-ups and blood tests. If you are worried or notice any new symptoms between appointments, tell your haematologist or clinical nurse specialist as soon as possible.

Making lifestyle changes to reduce your risk of complications can sometimes be challenging. You may find it easier to make changes as part of a group or with help from others. If you find it hard to make changes, tell your GP, practice nurse or haematology team. There is lots of support available to help you live a healthier lifestyle.

Everyone has their own way of dealing with the different feelings they experience. You may find it helpful to talk about things with family members, friends, or your specialist doctor or nurse.

Macmillan is also here to support you. If you would like to talk, you can:

• call the Macmillan Support Line for free on 0808 808 00 00. 
• chat to our specialists online. 

• visit the other cancers forum on our Online Community to talk with people who have been affected by PV – you can also share your experience and ask an expert your questions.

The organisations below also offer information and support: