What is myelofibrosis?
Myelofibrosis (MF) is a rare condition which affects approximately 1 in every 100,000 people. Myelofibrosis affects the bone marrow where red blood cells are made, disrupting the body’s production of red blood cells. Over time, myelofibrosis causes bone marrow to be replaced with fibrous scar-like material. This can eventually lead to a condition called progressive bone marrow failure.
Many people with myelofibrosis have symptoms which worsen over time, and some may eventually develop a more serious form of leukemia. In some cases, people will have myelofibrosis and not show any symptoms for long periods of time.
Causes and risk factors for myelofibrosis:
What causes myelofibrosis?
Myelofibrosis (MF) occurs when a genetic mutation occurs in blood stem cells. These cells are immature cells which can develop into any type of blood cell. As cells mutate, they pass their mutational characteristics onto other cells. Over time, this usually affects blood cell production, resulting in a lack of red blood cells in the bloodstream.
Nobody knows what causes these mutations to occur. However, there are several common risk factors associated with myelofibrosis (see below)
Risk factors of myelofibrosis:
A risk factor is a trait, condition or characteristic which is linked with an increased risk of myelofibrosis. Risk factors do not guarantee that myelofibrosis will occur, especially with the rarity of the cancer. Common risk factors include:
- Age – As you grow older, the risk of myelofibrosis increases. Myelofibrosis occurs most frequently in people over the age of 50.
- Other blood cell disorders – Polycythaemia (post-polycythaemic myelofibrosis) and essential thrombocythaemia (post-ET myelofibrosis) are two blood disorders which can cause myelofibrosis to occur.
- Exposure to toluene – Toluene is a chemical that is used in paint thinners, nail polish remover and some glues. Regular exposure to this chemical is linked with an increased risk of myelofibrosis.
- Exposure to benzene – Benzene is another widely used chemical which is found in crude oil and gasoline. It is used in the production of plastic materials, dyes and detergents. Regular exposure to benzene is linked with an increased risk of myelofibrosis.
- Exposure to radiation – Being exposed to radiation increases the likelihood of myelofibrosis occurring.
Signs and symptoms of myelofibrosis:
Below are some of the most common symptoms associated with myelofibrosis. If you notice any of these symptoms, it is best to be examined by a doctor as soon as possible. Early detection allows for more effective treatment of myelofibrosis.
Common symptoms of myelofibrosis include:
- Tiredness and fatigue – Myelofibrosis usually leads to a low red blood cell count (anaemia). This can cause tiredness, weakness and fatigue.
- Pain below the ribs – Myelofibrosis can cause an enlarged spleen, which often leads to pain around or below the ribs.
- Stomach pressure – An enlarged spleen will put additional pressure on the stomach. This can cause fullness and a loss of appetite. This can also cause pain and indigestion.
- Increased body temperature – Myelofibrosis can cause people to develop fevers, which can also lead to intense sweating during sleep (night sweats).
- Increased sensitivity to bleeding and bruising – A lack of red blood cells can lead to your body becoming more prone to bleeding or bruising.
How is myelofibrosis diagnosed?
The first step a doctor will usually take is to perform a physical examination. This will allow the doctor to assess your symptoms and determine if the spleen is enlarged.
Following a physical examination, a doctor will usually recommend a blood test. A sample of blood will then be checked for irregularities such as a low red blood cell count or changes in genes.
Bone marrow sample (biopsy):
A sample of bone marrow may be removed to be tested for signs of cancer. This process is called a biopsy. In many cases of myelofibrosis, this sample is removed from the hip bone (pelvis). Anaesthetic is usually used to numb the area, making the procedure painless. Once the sample is removed, it is taken to a lab and examined under a microscope. This allows doctors to determine if myelofibrosis is present.
Treatment options for myelofibrosis:
Doctors will aim to reduce symptoms of myelofibrosis by using common treatments for anaemia. This will encourage the body to produce more red blood cells. Common treatment options include:
- Blood transfusion – A blood transfusion is when blood is introduced to your bloodstream intravenously. This blood is provided by a donor whose blood type is compatible with your body. Blood transfusions are effective in treating severe anaemia and relieving symptoms.
- Anaemia medication – Your doctor may prescribe medications such as thalidomide, lenalidomide (Revlimid) and pomalidomide (Pomalyst) to increase the number of red blood cells in your bloodstream. These drugs may also be combined with steroid medications to improve effectiveness.
During chemotherapy, drugs are used to target and destroy cancer cells. For myelofibrosis, chemotherapy is often used to reduce the size of an enlarged spleen. This gives the patient relief from symptoms such as abdominal pain and stomach pressure. It also encourages the body to start producing red blood cells regularly again.
Chemotherapy can cause side effects. These include:
- Fatigue and tiredness.
- Increased risk of infections.
- Loss of hair.
- Nausea and vomiting.
During radiation therapy, high-powered beams and x-rays are used to target and destroy harmful cells. Radiation therapy may help reduce the size of the spleen and act as an alternative to spleen removal.
A splenectomy is a surgical procedure where the spleen is removed. Splenectomies are usually reserved for cases where the spleen is becoming too enlarged and leading to pain and other complications. This procedure may also be used for patients who are not responding well to other forms of treatment.
What support can we give for myelofibrosis?
Myelofibrosis is a rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. We provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other.
If you suffer from a rare cancer such as myelofibrosis, we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
You can help us with your donation:
Without a Ribbon is a charity that works hard to aid those who suffer from rare cancers. You can help our cause in a variety of ways:
Donations – Without a Ribbon is grateful for every donation received. Giving to Without a Ribbon helps us to provide ongoing support, organise the annual gathering and subsidise the costs of our Warriors attending these conferences.
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