Myelofibrosis occurs when the bone marrow of an individual is corrupted, causing the growth of abnormal fibrous tissue within the bone marrow and inhibiting the production of healthy blood cells. Over time, the bone marrow becomes scarred and is unable to produce blood cells at the rate needed by the body. The condition begins with the corruption of the bone marrow, causing the production of abnormal, immature blood cells. These blood cells have not yet matured into red blood cells, white blood cells, or platelets, and myelofibrosis can inhibit the production of one or all three types of cells. The signs and symptoms of the condition largely depend on which type of blood cell is primarily affected. The abnormal, immature blood cells replicate quickly and do not die when they should, causing the abnormal cells to accumulate in the blood stream. The abnormal cells eventually begin to crowd out the healthy blood cells that are needed by the body.
As the condition progresses, it can cause severe anemia due to the reduced amounts of red blood cells in the blood stream. Without an adequate supply of red blood cells in the system, parts of the body begin to suffocate from an inadequate oxygen supply. Anemia causes shortness of breath, fatigue, lethargy, and muscle weakness. In some cases, the individual affected by myelofibrosis will require regular blood transfusions to combat the effects of anemia and provide healthy red blood cells to carry oxygen through the body. The condition can also cause the liver and spleen of the patient to swell, as white blood cells begin to accumulate in these areas. This swelling causes the individual affected to experience an uncomfortable, full sensation in the abdomen and is a signature symptom of myelofibrosis. The activity of the abnormal blood cells also causes the scarring and growth of fibrous tissue within the bone marrow.
Researchers are unsure of why some individuals develop myelofibrosis and why some individuals do not. The condition seems to affect older individuals more often than younger individuals, with most of the diagnosed cases occurring in individuals over 50 years of age. Myelofibrosis can affect individuals of any age, although pediatric cases are extremely rare. The condition is an acquired mutation of the bone marrow and is not passed genetically from parent to child. Exposure to high levels of certain chemicals for long periods of time has also been linked to the development of myelofibrosis. These chemicals include benzene, which is used in high levels in a large number of industries, and a number of pesticides and herbicides. Previous exposure to radiation and chemotherapy has also been associated with the development of the condition. Research continues to discover the risk factors that can lead to the development of myelofibrosis.
There is no known way to prevent the development of myelofibrosis. In rare cases, the condition may be cured by bone marrow transplantation, but the risks of the procedure are so severe that the technique is not used for treating the condition very often. In most cases, the condition is given supportive treatment, which is not meant to cure the disorder but will alleviate the symptoms and improve the patient’s quality of life. The condition is considered rare, with around 1 in every 50,000 individuals developing myelofibrosis in their lifetime.
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