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This information is about chronic idiopathic myelofibrosis. It belongs to a group of conditions known as the chronic myeloproliferative diseases (CMPDs).
The chronic myeloproliferative diseases (CMPDs) are a group of conditions which affect how your bone marrow works. They involve over-production of one or more of the blood cells that are normally produced in the bone marrow. A small number of people with a CMPD will go on to develop a form of leukaemia.
The main types of chronic myeloproliferative disease are:
Chronic myeloid leukaemia (CML) is also a chronic myeloproliferative disease although it is usually dealt with as a separate condition.
This is the spongy material in the centre of some of our bones. It produces cells known as stem cells. These are immature cells that develop into three different types of blood cells:
Chronic idiopathic myelofibrosis is a condition in which scar tissue (fibrosis) develops in the bone marrow (myelo). Chronic means that the condition usually continues for many years, and the word idiopathic means the cause is unknown. Some people may have a faulty gene, called the JAK2 gene, that might have caused their condition.
Myelofibrosis is a rare condition which mainly affects people over 50. Its causes are unknown.
Myelofibrosis can develop when the bone marrow has been producing too many platelets (due to essential thrombocythaemia|), or too many red blood cells (due to polycythaemia vera|). It can also develop without any history of these other myeloproliferative diseases.
There are other conditions that can cause the bone marrow to become scarred and these are treated differently. Various tests and investigations will be done to determine the exact cause.
As the bone marrow becomes more scarred (fibrosed), it is less able to produce blood cells. To help compensate for this, the liver and the spleen begin to produce blood cells, and, as a result, these organs become enlarged. However, the liver and spleen are not as efficient at making blood cells as the bone marrow, and this can lead to low levels of blood cells in the body. When the spleen becomes enlarged it can 'hold on' to the blood cells and is involved in their destruction.
At first myelofibrosis may not cause any symptoms. Some people are diagnosed before they have any symptoms when they have a blood test taken for another reason.
As the bone marrow becomes less able to produce enough blood cells, the following symptoms may occur:
Myelofibrosis is usually diagnosed by a haematologist (specialist in blood disorders). The diagnosis may be suspected after the results of a blood test, known as a full blood count. This counts the number of the red cells, white cells and platelets in the blood. You may also have a blood test to check for the JAK2 gene. Other conditions can cause the bone marrow to become fibrosed. These need to be ruled out before a diagnosis of myelofibrosis can be made.
Your specialist may want to take a sample of marrow (biopsy) to examine under a microscope.
The sample is usually taken from the back of your hip bone (pelvis). You will be given an injection of local anaesthetic to numb the area. The doctor will then pass a needle through the skin into the bone, and draw a small sample of liquid marrow into a syringe (bone marrow aspirate).
After this, the doctor will take a small core of marrow from the bone (a trephine biopsy). Both samples will be looked at later under the microscope.
The test can be done on the ward or in the outpatients department. The whole procedure takes about 15–20 minutes. It may be uncomfortable when the marrow is drawn into the syringe, but this should only last for a few seconds.
You may feel bruised and have an ache for a few days after the test. This can be eased with mild painkillers.
If the bone marrow samples look normal, but your doctor still suspects you have myelofibrosis, they may want to take more samples from your pelvis. This is because, in the early stages of the disease, not all the bone marrow may be affected.
Other tests and investigations may also be done, such as scans and x-rays. Your doctor or nurse will explain these to you.
Treatment of myelofibrosis aims to control symptoms without causing too many side effects, and will depend on how the disease is affecting you. For example, if you have no symptoms you may not need any treatment for a while. In this case, your haematologist will monitor your condition regularly. If you develop any symptoms between appointments, let your specialist know.
If you have symptoms caused by a low red blood cell count (anaemia), you can be given transfusions of blood|. Usually it is possible to have a blood transfusion as a day-patient, but it may involve an overnight stay in hospital. Blood transfusions can be repeated as often as necessary.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It may be used to treat myelofibrosis, especially if the symptoms are severe. Chemotherapy| can help to reduce the size of the spleen and the liver, and can help to control other problems. It may also help to increase the number of blood cells. A number of different chemotherapy drugs can be used. Chemotherapy is often given to you as a day-patient but may sometimes mean an overnight stay in hospital.
One of the side effects of chemotherapy is to temporarily reduce the function of the bone marrow. For this reason chemotherapy is not suitable for everyone with myelofibrosis, and those that are treated need to be monitored very closely. Often the doses of chemotherapy are greatly reduced to help lower the risk of side effects.
Your doctor or nurse will explain which drugs you are to have, and any possible side effects.
Splenectomy This is an operation to remove the spleen. Your specialist may suggest that you have a splenectomy if you have a very low number of platelets, or if your spleen is enlarged and contains many blood cells as this will cause pain and discomfort. A splenectomy will improve your symptoms. This can be a risky operation for someone with myelofibrosis, and your specialist should discuss the pros and cons with you before you decide whether or not to have it.
Radiotherapy If it isn’t possible to remove the spleen, it may be possible to shrink an enlarged spleen using radiotherapy|. Radiotherapy is the use of high-energy rays to destroy cancer cells, while doing as little harm as possible to the healthy cells. Radiotherapy to the spleen can help to improve symptoms such as pain and a high platelet count. Unfortunately, the improvements in symptoms are temporary and may only last for a few months. Radiotherapy can cause side effects such as tiredness and skin irritation, although generally these are mild.
High-dose chemotherapy and stem cell transplant A small number of younger people with myelofibrosis may be able to have high-dose chemotherapy, followed by a stem-cell transplant|. The stem cells transplanted will usually come from a donor (an allogeneic transplant). Stem cells are blood cells at their very earliest stage of development. It is possible to collect them from the blood of a donor and store them ready to be transfused back into the person with myelofibrosis. Usually the donor is a brother or sister.
Before the stem cells are transplanted, you will be given high doses of chemotherapy to destroy your bone marrow. Once stem cells are transplanted, they start developing into mature blood cells. These eventually replace the cells your bone marrow produced before transplant, and before you got your blood disorder. A stem cell transplant involves staying in hospital for least a couple of weeks.
There are risks associated with this procedure, and as a result it is generally only suitable for people under 45 years. High-dose treatment with a stem cell transplant is not always successful.
If suitable, your specialist will discuss the possible risks of this treatment to help you decide whether or not to have a transplant.
Research into new ways of treating myelofibrosis is ongoing. Doctors use clinical trials| to assess new treatments. The drug thalidomide| is being investigated to determine if it is useful in the treatment of myelofibrosis. You may be asked to take part in this trial.
Thalidomide tablets may help to improve the number of red blood cells and platelets. Side effects can include: nausea|, headaches, constipation|, an increased risk of blood clots, and numbness or tingling in the hands and feet|.
Clinical trials are also looking at the use of drugs which affect the activity of the JAK2 gene (JAK2 inhibitors).
Before any trial is allowed to take place it must be approved by a research ethics committee, which protects the interests of those taking part. Before you enter a trial your doctor or research nurse must discuss the treatment with you so that you have a full understanding of the trial and what it involves. You may decide not to take part, or to withdraw from the trial, at any stage. You will then receive the best standard treatment available.
You will need to have regular check-ups and blood tests. These will probably be ongoing. If you have any problems, or notice any new symptoms in between these times, let your nurse or doctor know as soon as possible.
Everyone has their own way of dealing with their illness and the different emotions| they experience. Some people find it helpful to talk things over with family and friends or their doctor or nurse. You can also contact our cancer information nurse specialists| or another support organisation for more information and support.
This section has been compiled using information from a number of reliable sources, including:
Improving Outcomes in Haemato-Oncology. National Institute of Clinical Excellence, November 2003.
For further references, please see the general bibliography|.
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