Research and coping with polycythaemia vera (PV)

Polycythaemia vera (PV) is a type of rare blood cancer called a myeloproliferative neoplasm (MPN). These are conditions that cause an increase in the number of blood cells.

Research helps us find out more about conditions like polycythaemia vera.

Research into Polycythaemia vera

Polycythaemia vera is part of a group of conditions called myeloproliferative neoplasms. They also include:

• myelofibrosis
• essential thrombocythaemia

Researchers around the world are looking at better ways to treat polycythaemia vera and manage treatment side effects.

Go to Cancer Research UK’s clinical trials database if you are looking for a trial for polycythaemia vera in the UK. As this is rare cancer there may not be an open trial. Do talk to your doctor or specialist nurse, they may know of trials available in the UK for polycythaemia vera.

You can find a clinical trial on our clinical trials database. Click on the ‘recruiting’, ‘closed’ and ‘results’ tabs to make sure you see all the trials.

Some of the trials may have stopped recruiting people. It takes time before the results are available. This is because the trial team follow the patients for a period of time and collect and analyse the results. We include this ongoing research to give examples of the type of research being carried out.

Research and clinical trials

All cancer treatments must be fully researched before they can be used for everyone. This is so we can be sure that:

• they work
• they work better than the treatments already available
• they are safe

Research into diagnosis

Researchers are looking at ways to improve the diagnosis and treatment of all myeloproliferative neoplasms (MPNs).

JAK2 gene

The JAK2 gene makes a protein that controls how many blood cells the stem cells make. 

Some research suggests that it may be better to group MPNs depending on whether they are:

• JAK2 positive or
• JAK2 negative

This might improve targeted treatments in the future.

How do myeloproliferative neoplasms develop?

Researchers are looking in more detail at how myeloproliferative neoplasms develop. And to use this information to develop new treatments in the future. They think MPNs might develop because proteins called tyrosine kinases act as growth factors and tell cells to keep dividing. Trial teams are looking at white blood cells in samples from people taking part in a study. They are looking for tyrosine kinases proteins and for any features or damage that may make them keep on dividing.

Blood clots

UK trial teams are also looking more closely at the relationship between PV and blood clots.

Research into treatment

Trial teams are looking into new types of treatment to stop the JAK2 gene signalling to stem cells to make more blood cells. These are called JAK2 inhibitors.

Ruxolitinib is a JAK2 inhibitor. It works by slowing or stopping the growth of cancer cells. Some people with polycythaemia vera have ruxolitinib if another drug called hydroxycarbamide is not suitable for them.

One trial is comparing ruxolitinib with hydroxycarbamide for people with high risk PV. In this trial, people have ruxolitinib as their first treatment. Researchers want to find out: 

• how well ruxolitinib works as the first treatment
• more about the side effects
• how it affects quality of life

Doctors are also looking at giving ruxolitinib with another treatment called azacytidine. They want to find out whether they work better together or not. 

Another study is looking at how well givinostat works in people with myeloproliferative neoplasms, including polycythaemia vera. Givinostat is a drug that blocks particular proteins (enzymes) in the body. Blocking these enzymes may stop abnormal cells from growing and dividing. 

In another study, researchers are looking at a new type of targeted drug called a cancer growth inhibitor. They want to find how well it works in treating cancers that affect how well the bone marrow works. This includes polycythaemia vera. To do this the trial team needs to do a large clinical trial. But before doing this they need to find out: 

• what is the best dose 
• more about the side effects
• how acceptable it is to take by itself and with ruxolitinib

Treating an enlarged spleen

Many people with PV and other myeloproliferative neoplasms have an enlarged spleen. This can cause bloating and pain in the tummy (abdomen). A study is comparing a medicine called fedratinib with current available treatment. The study team wants to know if fedratinib works better at reducing the size of the spleen and improves the symptoms.

Outlook

Polycythaemia vera is often diagnosed later in life. Generally speaking, the outlook is good, if the condition is carefully monitored and treated as needed.

Some people with PV go on to develop a more aggressive disease. This might include progressing to myelofibrosis. This is where the bone marrow becomes scarred and less able to produce cells. Less commonly, people with PV can go on to develop acute myeloid leukaemia (AML).

Everyone is different. Your specialist will be able to give you a clearer idea of how things look in your situation.

Coping

Coping with a rare condition can be difficult both practically and emotionally. Being well informed about your condition and possible treatments can make it easier to make decisions and cope with what happens.

It can also help to talk to people with the same thing. But it can be hard to find someone with a rare condition. You can look at Cancer Research UK’s discussion forum - Cancer Chat. It is a place to share experiences, stories and information with other people who know what you are going through.

Organisations

There are organisations that also offer help and support. 

MPN Voice

MPN Voice provides information, community and advocacy for people with myeloproliferative neoplasms and their families or carers. They also have information for healthcare professionals.

Email: info@mpnvoice.org.uk 

Telephone: 07934 689 354 (Monday to Friday between 8am and 5pm)

Leukaemia Care

Leukaemia Care is a national blood cancer support charity, committed to ensuring that anyone affected by blood cancer receives the right information, advice and support. 

It provides a range of support services to patients and their carers, including information, support groups, patient and carer conferences, and nurse education days.

Freephone helpline: 08088 010 444 

Chat via WhatsApp on 07500068065

Blood Cancer UK

Blood Cancer UK funds research into leukaemia, lymphoma and myeloma. It also provides patient information booklets and leaflets that you can download or order from their website. 

Support line: 0808 2080 888 

Email address: support@bloodcancer.org.uk