Polycythaemia

Polycythaemia



Polycythaemia, also known as erythrocytosis, means having a high concentration of red blood cells in your blood.
This makes the blood thicker and less able to travel through blood vessels and organs. Many of the symptoms of polycythaemia are caused by this sluggish flow of blood.

Symptoms of polycythaemia

Not everyone with polycythaemia has symptoms – but many do.
Make an appointment to see your GP if you have persistent symptoms of polycythaemia. These include:
  • headaches
  • blurred vision
  • red skin – particularly in the face, hands and feet 
  • tiredness
  • high blood pressure 
  • dizziness
  • discomfort in the tummy
  • confusion
  • bleeding problems – such as nosebleeds and bruising
  • gout – which can cause joint pain, stiffness and swelling
  • itchy skin – especially after a bath or shower

When to seek immediate medical advice

Polycythaemia can cause blood clots. These put you at risk of life-threatening problems such as:
  • pulmonary embolisms – a blockage in the blood vessel that carries blood from the heart to the lungs
  • deep vein thrombosis (DVT) – a blockage that forms in the blood vessels in your leg before moving elsewhere in your body
Seek medical help immediately if you or someone you're with shows signs of DVT or a pulmonary embolism. These include:
  • pain, swelling, redness and tenderness in one of your legs
  • a heavy ache in the affected area
  • warm skin in the area of the clot
  • breathlessness
  • chest or upper back pain
  • coughing up blood
  • feeling lightheaded or dizzy
  • fainting
Polycythaemia also increases your risk of heart attack and stroke. Seek emergency medical help if you think that you or someone you're with is having a heart attack or stroke.

What causes polycythaemia?

Polycythaemia can be divided into several different types, depending on the underlying cause. In some cases, an underlying cause can't be identified.

Apparent polycythaemia

"Apparent polycythaemia" is where your red cell count is normal, but you have a reduced amount of a fluid called plasma in your blood, making it thicker.
Apparent polycythaemia is often caused by being overweight, smoking, drinking too much alcohol or taking certain medicines – including diuretics (tablets for high blood pressure that make you pee more).
Apparent polycythaemia may improve if the underlying cause is identified and managed. Stopping smoking or reducing your alcohol intake, for example, may help.

Relative polycythaemia

This is similar to apparent polycythaemia. It can happen as a result of dehydration.

Absolute polycythaemia

"Absolute polycythaemia" is where your body produces too many red blood cells. There are 2 main types:
  • primary polycythaemia – there's a problem in the cells produced by the bone marrow that become red blood cells; the most common type is known as polycythaemia vera (PV)
  • secondary polycythaemia – too many red blood cells are produced as the result of an underlying condition

Polycythaemia vera (PV)

PV is rare. It's usually caused by a change in the JAK2 gene, which causes the bone marrow cells to produce too many red blood cells.
The affected bone marrow cells can also develop into other cells found in the blood, which means that people with PV may also have abnormally high numbers of both platelets and white bloods cells.
Although caused by a genetic change, PV isn't usually inherited. Most cases develop later in life. The average age at diagnosis is 60.

Secondary polycythaemia

Secondary polycythaemia is where an underlying condition causes more erythropoietin to be produced. This is a hormone produced by the kidneys that stimulates the bone marrow cells to produce red blood cells.
Health conditions that can cause secondary polycythaemia include:

How polycythaemia is diagnosed

Polycythaemia can be diagnosed by carrying out a blood test to check:
  • the number of red blood cells in your blood (red blood cell count)
  • the amount of space the red blood cells take up in the blood (haematocrit level)
A high concentration of red blood cells suggests you have polycythaemia.
Polycythaemia is sometimes only discovered during a routine blood test for another reason.
Your GP may refer you to a haematologist (a specialist in blood disorders) for more tests, to confirm the diagnosis and to determine the underlying cause.
These may include:
  • a blood test to look for the changed JAK2 gene
  • an ultrasound scan of your tummy to look for problems in your kidneys

Treatments for polycythaemia

Treatment for polycythaemia aims to prevent symptoms and complications (such as blood clots), and treat any underlying causes.

Venesection (removing blood)

Venesection is the simplest and quickest way of reducing the number of red cells in your blood. It may be recommended if you have PV, a history of blood clots, or symptoms suggesting your blood is too thick.
Venesection involves removing about 1 pint (half a litre) of blood at a time, in a similar way to the procedure used for blood donation.
How often this is needed will be different for each person. At first, you may need the treatment every week, but once your polycythaemia is under control you may only need it every 6 to 12 weeks or less.

Medicine to reduce red blood cells

In cases of PV, medicine may be prescribed to slow down the production of red blood cells.
Many different medicines are available and your specialist will take into account your age and health, response to venesection and red blood cell count when choosing the most appropriate one for you. Examples include:
  • hydroxycarbamide – this medicine is taken as tablets every morning and is generally tolerated well. But you should not take it if you're pregnant or trying to become pregnant
  • interferon – this medicine is given by injection into the abdomen or thigh 1 to 3 times a week. You can inject it yourself at home once you've become familiar with how to do it. Interferon has the advantage that it can be taken in pregnancy, but it may cause unpleasant side effects, such as hair loss and flu-like symptoms

Medicine to prevent blood clots

If you have PV, daily low-dose aspirin tablets may be prescribed to help prevent blood clots and reduce the risk of serious complications.
You may also be offered treatment with low-dose aspirin if you have apparent or secondary polycythaemia and another health problem affecting your blood vessels, such as coronary heart disease or cerebrovascular disease.

Treating and preventing other symptoms

Some people may also need treatment for any other symptoms or complications of polycythaemia they have, or for any underlying cause of the condition.
For example, you may be given medicine to help relieve itching or manage COPD. Read more about:

Lifestyle changes that help

As well as improving some cases of apparent polycythaemia, making healthy lifestyle changes can also reduce the risk of potentially serious blood clots for people with all types of polycythaemia.
Having polycythaemia means you're already at high risk of a blood clot, and being overweight or smoking only increases this risk.
You may find the following advice and information helpful:

Outlook for polycythaemia

The outlook for polycythaemia largely depends on the underlying cause.
Many cases are mild and may not lead to any further complications. However, some cases – particularly cases of PV – can be more serious and require long-term treatment.
If well controlled, polycythaemia should not affect your life expectancy, and you should be able to live a normal life. However, people with PV can have a slightly lower life expectancy than normal due to the increased risk of problems, such as heart attacks and strokes.
PV can also sometimes cause scarring of the bone marrow (myelofibrosis), which can eventually lead to you having too few blood cells. In some rare cases, it can develop into a type of cancer called acute myeloid leukaemia (AML).
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