The Truth About Myelofibrosis: A Serious Condition
Myelofibrosis is a serious condition that affects bone marrow cells and causes them to become fibrous and scarred
. Bone marrow plays a vital role in production of blood cells. Therefore, myelofibrosis adversely impacts blood cell production. The number of red blood cells falls significantly, while white blood cell production increases. However, the white blood cells produced are malformed and often ineffective in fighting off infection. Platelets which are instrumental in clotting also fluctuate during the progression of the disease.
The onset of the disease is often gradual and the patient may not display significant symptoms in the early stages. Anemia as a result of low red blood cell production is often one of the earliest signs that something is amiss. As the bone marrow becomes less effective the spleen and liver take on an increasing role in production of blood cells. Consequently, most patients develop splenomegaly (enlarged spleen) and a significant proportion have hepatomegaly (enlarged liver).
Other symptoms include:
Fatigue as a result of anemia.
Shortness of breath.
Painful bones especially in the lower legs.
Fever
Night sweats.
Susceptibility to infection from compromised immunity as a result of ineffective white blood cells.
Bruising and bleeding easily.
Pain and tightness below left ribs due to enlarged spleen.
Diagnosis would be made based on physical examination and confirmed by blood tests which reveal tear-drop shaped red blood cells and bone marrow tests showing increased fibrosis.
Myelofibrosis can occur at any age. However, it is most common among people aged over 50. There is no cure and treatment is focussed mainly on alleviating the effects of the symptoms and slowing down its impact on other organs.
Speed at which the disease progresses varies considerably between individual patients with survival ranging from one to fifteen years with an average of around five years.
Complications that can occur include:
Increased pressure in the liver. Enlarged spleen increases blood flow through the portal vein into the liver and can increase the risk of rupture of smaller blood vessels in the stomach and other internal organs. Liver failure is another complication in later stages of myelofibrosis.
Enlarged spleen also results in more platelets being sequestered in the spleen and drawn out of circulation. Reduced platelet count increases clotting times and also increases risk of bleeding and bruising.
Mutated white blood cells leave the immune system unable to combat infection and many opportunistic infections may take hold.
Some patients may also develop an acute form of Leukemia which progresses rapidly.
Causes of Myelofibrosis are not known for certain although there are some factors which appear to be related including:
Exposure to certain toxins such as Benzene and Thorium Dioxide.
It has also been linked with some genetic abnormalities. However, there is no conclusive evidence that it is congenital. In fact the preponderance of evidence seems to suggest that the cell mutation is acquired during the patient's lifetime.
Given that the cause is not known no preventative measures can be prescribed except to avoid exposure to the toxins suspected of having a part to play in development of this disease.
by: Ryan Round
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