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Myelofibrosis

 

What is it?

Myelofibrosis is a condition where the bone marrowThe tissue in the hollow areas of bones. This produces the blood cells becomes scarred. As the bone marrowThe tissue in the hollow areas of bones. This produces the blood cells becomes scarred it cannot to its job properly, which is to make blood.

 

What causes it?

The bone marrowThe tissue in the hollow areas of bones. This produces the blood cells produces too many cells. These cells release signals which cause the bone marrow to become scarred. The scarring gets worse and prevents the bone marrow from doing its job of producing the different cells found in blood.

 

What are the symptoms?

A small percentage of patients have no symptoms but nearly all patients have abnormally large spleensAn organ in the left upper abdomen which removes old red blood cells from the blood. This is felt a lump underneath the ribs on the left side. In myelofibrosis the spleenAn organ in the left upper abdomen which removes old red blood cells from the blood can become very large and reach down to the belly button. The liverAn organ in the right upper abdomen which has many functions including the removal of toxic substances from the blood and production many vital nutrients. is often enlarged as well, this may be felt as a lump underneath the ribs on the right side.

As the bone marrowThe tissue in the hollow areas of bones. This produces the blood cells does not produce enough red blood cells patients can become anaemic, so they can feel tired and short of breath. Also not enough white cells are produced and patients can get infections. Patients can also get nose bleeds and bruising as there are not enough platelets made to make the blood clot as normal. Patients can also generally feel unwell with night sweats, weight loss, fevers and feeling tired.

 

How is it diagnosed?

There are clues on blood tests and when blood cells are looked at under the microscope. However, to be sure of the diagnosis a bone marrow biopsy is needed. The bone marrow The tissue in the hollow areas of bones. This produces the blood cells has a very characteristic appearance and enables the diagnosis to be made.

 

How is it treated?

Most of the treatment aims to reduce the effects of the myelofibrosis rather then to cure it. Some cases need close monitoring in clinic only, whereas others require chemotherapy. If a patient becomes anaemic then tablets or a blood transfusion is given. Patients are also at risk of infections which need early treatment.
Myelofibrosis can unfortunately switch into acute leukaemia towards the end stages of the disease.

 

Further information

Myelofibrosis at PubMed Health (US National library of medicine)

 

 

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Written by: Dr T Rider

Editor: Dr J Newman

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