This type of bone marrow cancer affects your body's ability to produce blood cells. It's a type of cancer known as myelofibrosis (MF), which is part of a group called myeloproliferative neoplasms (MPNs). It causes your bone marrow cells to stop developing and functioning properly, resulting in fibrous scarring. There are two types of MF: primary, in which it occurs on its own, and secondary, in which it results from another condition. There are other MPNs that can progress to MF as well. Some people are asymptomatic for years, while others develop symptoms due to scarring in the bone marrow.
Causes of Myelofibrosis
As a result of mutations (changes) in the DNA of blood stem cells, myelofibrosis occurs. Red blood cells, white blood cells, and platelets are all formed from stem cells, which can replicate and divide into these specialized cells. There is no clear cause for this mutation. As the mutated cells replicate and divide, they pass along the mutation to new blood cells. Eventually, the mutated cells overtake healthy blood cells produced by the bone marrow. Generally, myelofibrosis causes anemia due to a lack of red blood cells, as well as an excess of white blood cells and varying levels of platelets. A person with myelofibrosis suffers from scarring of the normally spongy bone marrow. There are several specific gene mutations associated with myelofibrosis. The most common mutation is the Janus kinase 2 (JAK2). CALR and MPL are other less common mutations. You can determine your prognosis and treatment based on whether these gene mutations are associated with your myelofibrosis.
Symptoms of Myelofibrosis
Many people don't notice symptoms at first of myelofibrosis, but as it progresses and interferes with blood cell production, symptoms may include:
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Tiredness
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Breathing difficulties
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Easy bruising or bleeding
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Under your ribs, you feel pain or fullness on your left side
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Sweating at night
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The fever
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Pain in the bones
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Weight loss and loss of appetite
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Bleeding gums or noses
Diagnosis of Myelofibrosis
A complete blood count (CBC) usually shows myelofibrosis. It is common for people with MF to have low levels of red blood cells and unusually high or low levels of white blood cells and platelets. Depending on the results of your CBC test, your doctor might also perform a bone marrow biopsy. In this case, your doctor will take a small sample of your bone marrow and examine it closely for signs of MF, such as scarring. Your symptoms or CBC results may also require an X-ray or MRI scan to rule out any other possible causes.
Treatment of Myelofibrosis
There are several treatment options available for MF, depending on the type of symptoms you have.
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A variety of drugs can be used to treat MF, including:
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Epoetin alfa and darbepoetin alfa are examples of growth factors for anemia, while filgrastim and pegfilgrastim are examples of growth factors for low white blood cell counts.
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Demethylation agents: These are drugs that help bone marrow cells mature and function normally by modifying their DNA. Examples include azacitidine and decitabine.
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One example of an immunomodulator is lenalidomide, which is used for MFs with a deletion of part of chromosome 5 (del(5q)).
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Examples include anti-thymocyte globulin and alemtuzumab, which suppress the immune system and prevent it from attacking bone marrow cells.
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The purpose of blood transfusions is to replace low or missing blood cells with blood or blood components from a donor. Anemia, bleeding, or infection may require red blood cell transfusions, platelet transfusions, or white blood cell transfusions for people with MF.
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This procedure involves replacing the diseased bone marrow with healthy bone marrow from a donor. Although this is the only cure for MF, it is also a complex and risky procedure that is not suitable for everyone. It is usually reserved for younger patients with high-risk MF, who have a compatible donor.
Prevention of Myelofibrosis
In order to reduce the risk of developing MF or its complications, some lifestyle changes and precautions may help. These include:
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Smoking cessation
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Bone marrow can be damaged by chemicals or metals
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Maintaining a healthy diet by eating a balanced and nutritious diet
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Regular exercise
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Keeping a healthy weight
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Infections can be prevented by washing hands frequently
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If you experience any symptoms of MF, seek medical attention immediately
