• What is MF?
  Myelofibrosis (MF) is one of the myeloproliferative disorders (MPDs). It is also known as agnogenic myeloid metaplasia or meylofibrosis with myeloid metaplasia.  In this condition the bone marrow (the place in the body that produces blood cells) becomes scarred or fibrosed. This scarring occurs because there are too many cells (fibroblasts) in the marrow. Having too many fibroblasts in the marrow can in turn stop the bone marrow from making blood cells.

MF usually occurs in people over 60 years old. MF can be primary or idiopathic (meaning the cause is unknown). Secondary MF develops as a result of another condition, usually from other MPDs such as Essential Thrombocythaemia (ET) or Polycythaemia Vera (PV). Sometimes secondary MF can occur as a reaction to other disorders such as auto-immune/inflammatory conditions or cancers like Hodgkin’s Disease.

As MF progresses, a patient's blood counts may fall. The spleen will enlarge as it tries to help the marrow to make blood cells, and sometimes the liver can get bigger too. This happens because when each of us were babies growing in the womb, our liver and spleen made blood cells for the first two months of life, before the bone marrow started to work properly. As the spleen gets bigger with MF, it can become very large, taking up a lot of space in your abdomen.

When the spleen gets this big, it acts as a reservoir and pools the blood, so fewer blood cells can circulate and the blood counts fall even lower.

• How is MF diagnosed?
  The diagnosis of MF usually includes finding a low total blood count (pancytopenia) and a large spleen. Sometimes in the early stages the bone marrow is overactive and makes too many blood cells. This is before the fibrous scarring begins to become very prominent and damages the bone marrow. The haematologist (blood diseases doctor) will examine the symptoms, look for features of the disease and do the following tests:
  
  
  • Blood tests
  • Ultrasound scan
  • Bone marrow test

The bone marrow test involves taking a small sample from the pelvic bone. It is usually done under a local anaesthetic in the hospital day unit as a day case. This test confirms the diagnosis and a special stain (reticulin) tells us how severe the fibrosis is.

• What is bone marrow?
  Bone marrow is a spongy substance that runs along the inside of your long bones and acts as a "factory" for making blood cells. By looking at a sample of marrow under the microscope, the haematologist will get a good picture of your blood-making mechanism.
  
  
• What is involved in a bone marrow test?
  Your doctor with usually take a sample of the bone marrow from the back of your pelvis bone. You will generally be asked to lie on your side with your legs curled up.

The area where the sample is to be taken will be cleaned and numbed by an injection of local anaesthetic - this can sometimes cause a stinging sensation which only lasts a few seconds. The doctor will insert a needle into the anaesthetised area. You may feel a slight "pulling" sensation as the sample is taken.

For some people, a second sample called a trephine biopsy is required. This involves collecting a "core" of the bone. At this time you may feel an uncomfortable pushing sensation, but it will only last a few moments.

The whole procedure usually takes 20-30 minutes. The site will be covered with a small dressing and you will be asked to lie on your back for a few minutes.

The dressing needs to stay in place for 24 hours and it is best to avoid soaking in the bath during this time. You may experience slight discomfort in the area as the local anaesthetic wears off, which can be helped by taking a simple painkiller such as paracetamol.

• What are the symptoms of MF?
  Initially there may not be any obvious symptoms and their doctor may make a diagnosis of MF based on abnormal findings in the blood. There may also be symptoms caused by low haemoglobin (anaemia); these symptoms include tiredness, lethargy, shortness of breath, palpitations and chest pain. In addition, bruising or problems with bleeding sometimes occur due to the low platelet count.

As the spleen gets bigger it can cause abdominal pain, decreased appetite, feeling of fullness in the abdomen after small meals and sometimes shoulder tip pain as well.

As the disease progresses there will be weight loss, sweats, fevers and fatigue. There also may be more infections because of the fall in white cell counts.

• Which cells does MF affect?
  There are three main cells that doctors focus on in the blood of MF patients:
  • Red cells – these contain haemoglobin and carry oxygen around the body. As these fall you may become anaemic and need to have a blood transfusion.
  • Platelets – these help with producing blood clots. As these fall problems with bleeding may develop and you may need to have a platelet transfusion.
  • White cells – these help to fight infections. As they fall in number or do not function normally there is a risk of infections. Antibiotics may be needed to prevent the infection from getting worse.
    
    
• What is the treatment for MF?
  The main aims of the treatment are to control the symptoms of the disease, either to address low blood counts or to manage the size of the spleen. There are also treatments that may be considered to control the disease as it progresses.
  
  
• What is the prognosis?
  MF is a progressive disorder, which is irreversible in most cases. The prognosis depends on age, the results of the blood and bone marrow tests, the symptoms and whether the cause of the myelofibrosis is primary or secondary (as a result to another disorder). The haemoglobin level, the white cell count, age and bone marrow cytogenetic (chromosome) results are all taken into account in assessing the disease. The prognosis is very variable and can range from over a year to over fifteen years. You need to discuss your case with your haematologist, as this is very individual.

If you'd like to read more detailed information about myelofibrosis (MF), please go to the MF main page