High red blood cells (primary polycythaemia vera) Pūtau toto whero teitei
Primary polycythaemia vera is a rare disease caused by your bone marrow making too many red blood cells. It can be diagnosed at any age, but the average age for diagnosis is 60. It is more common in men than in women.
Cause of primary polycythaemia vera
The cause of primary polycythaemia vera is usually a change (mutation) in a gene called JAK2. It is usually a genetic change that happens in your lifetime. It is not passed down from a family member.
It is not contagious and you cannot pass it on to others.
Symptoms of primary polycythaemia vera
Primary polycythaemia vera develops slowly and may not cause symptoms for many years. It is often picked up on a routine blood test.
You may have:
- headaches
- weakness or dizziness
- fatigue
- red or itchy skin
- changes in vision
- excessive sweating
- red or burning hands or feet (erythromelalgia)
- excessive bleeding or bruising
- weight loss
- bloating
- abdominal pain due to an enlarged spleen
- gout (painful inflammation in the joints).
Complications of primary polycythaemia vera
If you have primary polycythaemia vera, you are more likely to get blood clots (thrombosis). This is because you will have more red blood cells and platelets than people who do not have it.
Thrombosis can include:
- stroke
- heart attack
- a clot in the lungs (pulmonary embolism)
- a clot in the leg (deep vein thrombosis).
Polycythaemia vera can progress to more aggressive blood conditions such as myelofibrosis and acute myeloid leukaemia. These are rare and you would be referred to a specialist for treatment.
Diagnosing primary polycythaemia vera
You will usually be diagnosed with primary polycythaemia vera after a routine blood test finds high numbers of red blood cells. Sometimes you may also have high numbers of white blood cells and platelets.
You might need further tests to rule out other blood conditions. In some cases your healthcare provider may request a bone marrow biopsy (sample).
Treatment for primary polycythaemia vera
There is no cure for primary polycythaemia vera but you can have a good quality of life if you manage the symptoms. The aim of treatment is to reduce the chance of blood clots by:
- taking out some of the blood in your veins to reduce the number of blood cells (venesection or phlebotomy) — you may need this to be done every 1 to 2 weeks at first
- taking medication to reduce the risk of clotting, or to reduce specific symptoms — sometimes you might take medication to reduce your red blood cell count.