Myelofibrosis is dangerous disorder of the bone marrow. It is a condition that interrupts the normal blood cell production and causes broad scarring of the bone marrow that eventually results in anemia, fatigue, weakness and sometimes spleen and liver enlargement. Myelofibrosis is actually a rare type of chronic leukemia. It belongs to the group of myeloproliferative disorders. Some myelofibrosis cases may not show any signs of the disease even for years. However, most of the cases become worse with time, and few can eventually develop into more serious form of leukemia. The treatment options focus on relieving the symptoms.
Myelofibrosis is a slowly developing disease that usually doesn’t cause symptoms to appear in the beginning stages, but as the interruption of the production of blood cells increases, symptoms such as the following may appear:
- Feeling tired and weak
- Shortness of breath
- Pale skin
- Bruising and bleeding easily
- Excessive sweating over night
- High temperature
- Pain in the bones
- Recurrent infections
The blood stream cells, red blood cells, white blood cells and platelets, are able to divide and replicate into multiple specialized cells that consist the blood stream. When a genetic mutation develops in the blood stem cells, myelofibrosis appears. It is not known what causes this mutation to happen. When the mutated blood stem cells divide and replicate they pass on the mutation to the newly formed cells, so the number of the defective cells rises and seriously affect the production of blood. Usually the result is lack of red blood cells and an surplus of white blood cells and platelets. The lack of red blood cells causes the characteristics and symptoms of anemia to appear. The bone marrow that should normally be soft anf spongy, becomes scarred. The mutated gene that appears in most of the myelofibrosis cases is sometimes referred to as JAK2.
The cause for myelofibrosis is not known, but some factors are known to increase the risk. They are:
- Age-people in the 50s or 60s are at greater risk
- Another blood cell disorder such as thrombocythemia or polycythemia vera
- Exposure to industrial chemicals like toluene and benzene
- Exposure to high levels of radiation
Some complications that can arise from myelofibrosis can be:
- Increased pressure on the blood flowing into the liver
- Abdominal an back pain
- Growths in other body areas
- Excessive bleeding
- Pain in the bones and joints
- Acute leukemia
If symptoms and visible signs of anemia aren’t noticeable, the person affected with myelofibrosis may not need treatment. However, the doctor should monitor the condition for any changes, symptoms and progression of the disease.
There are some research studies that work on developing a medicine that will target the JAK2 gene mutation which is thought responsible for developing myelofibrosis.
If the myelofibrosis causes anemia, some of the following treatments should be considered:
- Blood transfusion
- Androgen therapy
- Thalidomide and related medications
When the enlarged spleen is causing further complications, the following treatment options may be suggested by the doctor:
- Splenectomy-surgical removal of the spleen
- Radiation therapy
Another treatment option can be stem cell transplantation from a suitable donor. This is actually the only one treatment that has potential to cure myelofibrosis, but it carries a high risk of fatal side effects.