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What is it about ?
Myelofibrosis is a rare condition of the bone marrow. New blood cells are formed in the bone marrow. Our blood cells have a limited lifespan. That is why they are continually replaced.
In myelofibrosis, there is an overgrowth of certain cells in the bone marrow and an invasion of the bone marrow by connective tissue. This then prevents normal production of new blood cells.
It is not clear what exactly causes myelofibrosis. In 8 out of 10 people, there is a genetic mutation.
In whom and how often does it occur?
Myelofibrosis is a rare condition. It occurs in less than one in 100,000 people per year, and mostly affects people between the ages of 40 and 70. Men are as affected as women. The average survival time is 5-7 years, but it can vary widely, ranging from 1 year to over 20 years.
How to recognize it?
The course of the disease is usually very slow. The number of blood cells decreases. In response, the spleen tries to compensate for this lack by producing blood cells: the spleen swells and is painful.
At first, myelofibrosis usually doesn’t trigger many symptoms. There is mainly fatigue and pallor, due to anemia. The swelling of the spleen results in an upset stomach, loss of appetite, and a rapid feeling of having eaten enough (because the spleen is pushing on the stomach).
During the first years of the disease, platelet production may increase, increasing the risk of blood clots forming. The more connective tissue invades the bone marrow, the more marked the complaints: bone pain, leg cramps, weakness, shortness of breath on exertion, weight loss, gout, etc.
The disease also develops deficiencies in white blood cells and platelets, which increases the risk of infections and bleeding.
How is the diagnosis made?
If the doctor sees swelling (enlargement) of the spleen and / or liver during their physical examination, they will have a complete blood test. If in doubt, he asks for an ultrasound.
If he suspects myelofibrosis, a bone marrow exam is needed to confirm the diagnosis: under local anesthesia, some bone marrow is aspirated from the bone with a large needle. This procedure is sometimes difficult to perform because the bone marrow is filled with connective tissue. In this case, a bone marrow biopsy is done. This examination is carried out in the context of hospitalization.
What can you do ?
If you feel tired or have other symptoms that correspond to anemia, you must see the doctor. Also if you have pain in the upper stomach on the left or if you have a feeling of heaviness in the same place.
What can your doctor do?
To date, no treatment has made it possible to cure myelofibrosis or to delay its progression. Treatment consists of relieving the symptoms. It is determined by a specialist in internal diseases (internist) or blood diseases (hematologist). Regular monitoring is necessary to follow the progression of the disease.
The type of treatment depends on the symptoms, the stage of the disease and the general condition of the person.
Anemia is treated with the administration of erythropoietin (EPO) and blood transfusions. Usually, antibiotics are given to treat infections. Chemotherapy and immunotherapy reduce the size of the spleen and prevent excessive production of blood cells. The spleen is surgically removed (removal of the spleen, splenectomy) only in exceptional cases. Aspirin reduces the risk of thrombosis.
A bone marrow or stem cell transplant is the only possible cure.
Want to know more?
- Ultrasound here Where to find on this page of Cliniques St Luc UCL
- Bone marrow puncture – Foundation against cancer
- EPO – CBIP – Belgian Center for Pharmacotherapeutic Information
- Chemotherapy – Foundation against cancer
- Immunotherapy – Foundation against cancer
- Receiving a stem cell transplant – canadian cancer society
Source
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