FAQs: Your Questions Answered...
Q. What types of conditions are known as myeloproliferative neoplasms (MPNs)?
MPNs (or myeloproliferative neoplasms) are a group of closely related chronic conditions. They are all stem cell disorders that develop when your body produces too few blood cells or too many mature blood cells too quickly. There are four main types of MPNs that add up to approximately 95% of all cases: essential thrombocythaemia (ET); myelofibrosis (MF); polycythaemia vera (PV) and chronic myeloid leukaemia (CML).
- PV occurs when too many red blood cells are made.
- ET occurs when too many platelets are made.
- MF occurs when bone marrow – the soft, fatty tissue inside your bones that produces stem cells – is replaced by fibrous (or scar) tissue and is not able to make enough blood cells.
- CML occurs when an abnormality in the stem cells of the bone marrow causes a massive increase in the number of white blood cells.
- CML is associated with the presence of the Philadelphia chromosome whereas ET, MF and PV are known as Philadelphia chromosome-negative MPNs due to its absence.
Q. What symptoms am I likely to experience?
Although ET, PV and MF are different diseases, they do share many of the same symptoms. One study asked over 1,000 patients with ET, MF or PV to report their most significant symptoms:80.7% - significant fatigue 52.2% - itching 49.2% - night sweats 43.9% - bone pain 13.7% - fevers 13.1% - undesired weight loss
Each MPN is also associated with its own list of characteristic symptoms. Find out more by clicking on a condition below, but remember that all individuals with an MPN, experience different symptoms at different times. MF » PV » ET »
Q. How are myeloproliferative neoplasms (MPNs) caused?
As time goes on we are learning more about MPNs and their causes. While we currently do not know the exact cause, recent research found that about 50% of people with essential thrombocythaemia (ET) or myelofibrosis (MF) and 97% of people with polycythaemia vera (PV) have a change (mutation) in a protein that regulates blood cell production (this protein is known as JAK2).
Between 5 and 10% of people with MF have a mutation in another gene called MPL, while another 23% have another more recently discovered mutation called calreticulin. People are not born with these mutations but acquire them during their lives. Some researchers believe MF can be triggered by a viral infection, exposure to toxins or exposure to radiation.
Q. How rare are myeloproliferative neoplasms (MPNs)?
Before getting your diagnosis, you probably hadn’t heard the terms myeloproliferative neoplasm or MPN, or met anyone with the condition. That’s because MPNs are considered rare diseases. A disease is generally regarded as rare if it affects less than 50 people in 100,000.
Annually there are:
- Between 1.5 and 3 cases of ET per 100,000 people.
- 2 cases of PV per 100,000 people.
- Between 0.4 and 1.5 cases of MF per 100,000 people.
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Q. Will my children inherit this disease?
MPNs are not generally inherited diseases so you can’t pass it on to your children and you didn’t get it from your parents. Recently, researchers have discovered that these diseases may be caused by gene mutations (changes in DNA). Although these are acquired (you’re not born with them) this may explain why some families do demonstrate a clear predisposition.
Q. How long will I live?
Your individual situation and health history, as well as the ways you respond to treatment, can all affect your prognosis. Your haematologist will be able to provide you with a more accurate picture.
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Q. Do I have cancer?
Until recently there was some debate about whether myeloproliferative neoplasms (MPNs) such as ET, PV and MF are cancers or not because the word ‘neoplasm’ (new growth) is a term that doctors use both for cancers (malignant neoplasms) and non-cancerous tumours (benign neoplasms). Now, however, because MPNs are characterised by uncontrolled cell growth, most doctors and cancer organisations do classify them as blood cancers. Whatever they’re called, though, remember that symptoms and prognosis can vary widely. Your specialist will advise you based on your individual circumstances.
Q. What lifestyle changes should I consider?
If you have an MPN, taking care of yourself by adopting a healthy lifestyle can reduce your chances of suffering a clot, keep your immune system strong, increase your energy and help you feel as mentally and physically well as possible. Try these positive lifestyle choices:
Activities such as sports, walking, swimming, yoga or tai chi have a host of wellbeing benefits. Your doctor will tell you about your platelet, red cell and white cell levels and whether it is safe to exercise.
Boost energy by eating more vegetables and fruit, switching animal fats for healthy fats, choosing lean sources of protein and reducing intake of sugar, white flour, trans-fats and processed foods.
Quitting smoking is one of the most important things you can do to improve your wellbeing. If you need help, talk to your doctor.
Watch your alcohol intake
If you drink alcohol, it’s best to drink in moderation, and drink a glass of water for each glass of alcohol.