Polycythaemia vera (PV)
Polycythaemia vera (PV) treatment and side effects
In this section we talk about the specific treatments used for polycythaemia vera (PV).You might also want to read our general information about how blood cancer treatment is planned and managing your treatment.
We also have some important information on staying safe if you’ve got blood cancer, covering things like risk of infection and vaccinations.
The aim of treatment for PV is to reduce the risk of getting thrombosis by reducing the number of red blood cells in your blood. You should also be monitored for cardiovascular risk factors such as diabetes, high cholesterol, high blood pressure and smoking, and these should be addressed as effectively as possible.
Although currently PV can’t be cured, it can be kept under control to reduce the symptoms and complications it may cause.
The treatment you receive for PV will depend on the following factors:
- how high the packed cell volume (PCV) is
- your age
- the type of blood cell most affected.
If you’ve been diagnosed with PV and don’t have any symptoms, you may not need to start treatment for a while. Instead, you'll be monitored with regular check-ups and blood tests; this is known as 'watch and wait'. While this might seem strange, there’s no evidence to show that treating people with no symptoms has any impact on their outcome. It also means you don’t get any side effects from unnecessary treatment.
If you do have symptoms, your treatment will include some or all of the following options:
This is one of the simplest and quickest ways to reduce the number of red blood cells in your blood and make your blood thinner. This is also known as blood letting or phlebotomy. It involves taking around a pint (half a litre) of blood from you. This may be done once a week initially and then repeated as often as needed.
You may feel faint after the blood is taken, so replacement fluid can be given at the same time to help with this.
If you have PV and other risk factors such as previous clots, diabetes and high blood pressure, you may be at an increased risk of blood clots so your doctor may recommend you take low-dose aspirin regularly.
If you need to take painkillers for any other reason at the same time, ask your doctor what you can safely take with the aspirin. If you are already taking an anticoagulant such as warfarin, your doctor may decide you do not need aspirin as well.
If your platelet count is high or you have other symptoms such as weight loss or sweats, you may be given tablets called hydroxycarbamide (or hydroxyurea) to take. This is a mild form of chemotherapy and works by directly preventing the production of red blood cells. Hydroxycarbamide is the most common chemotherapy drug used to treat PV.
You might get some side effects from this treatment. These might include more infections than normal, diarrhoea or constipation. Your healthcare team will be able to help you manage side effects like this.
Hydroxycarbamide is a very safe treatment. However, there’s a theoretical risk that it may increase the risk of PV transforming into acute myeloid leukaemia (AML) if it’s used as a long term treatment. For many people, the benefits of the treatment usually outweigh any potential small risk.
Interferon is another drug you may take if your platelet count is high or you have symptoms such as weight loss or sweating. Interferon is an injection that reduces the rate at which blood cells are made. It’s not thought to carry the same risk of developing leukaemia as hydroxycarbamide when used in the long-term, and is the preferred choice for younger patients. However, many people find the short-term side effects unpleasant and not everyone can tolerate interferon therapy.
Possible side effects you may get while being treated with interferon include:
- flu-like symptoms
- mood swings
Ruxolitinib is a type of drug known as a JAK2 inhibitor.
In Scotland, ruxolitinib can be offered to people who can't hydroxycarbamide.
In England, Wales and Northern Ireland, this drug hasn't been approved for routine use on the NHS for people with PV, but it may sometimes be offered.
Busulfan is another chemotherapy drug. It may be used when hydroxycarbamide isn’t appropriate or isn’t working. It’s given as a tablet. Side effects can include lung tissue damage or reduced numbers of red blood cells, white blood cells or platelets in the blood. Busulfan can also increase the risk of leukaemia developing, so it’s only used when doctors believe the benefits of treatment outweigh the risks.
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