Thứ Hai, 13 tháng 1, 2014

Myelofibrosis

Myelofibrosis Myelofibrosis is the condition of chronic leukemia or blood cancer that seriously affects the regular blood cell formation in the body. This disease produces scarring on the bone marrow causing fatigue, weakness and anemia and it also affects the liver causing enlarged liver and spleen. Myelofibrosis either occurs in the primary form or in the secondary form (adverse effect of bone marrow disorder). In the long run this disease will make your health condition worsen leading to serious type of leukemia. Treatments are available to get rid of symptoms and the affected person can live for long years without any serious symptom.


Symptoms :


This disorder progresses very slowly and hence the affected individual will not show any sign in the initial stages. As the disease develops it will show symptoms like enlarged liver, fatigue, weakness, extreme tiredness, anemia, and feeling of fullness on the left side, excessive bleeding, getting bruises easily and getting frequent infections. The person will have high fever and profuse sweating during night time while sleeping.


Causes :


Myelofibrosis is caused due to certain type of genetic mutation. But it is not known what causes this genetic mutation in the blood cells. Once the mutation occurs it produces defective stem cells which would then divide and replicate to produce fresh defective stem cells. At one point more number of mutated cells remains on the body which would affect the level of normal blood cells. Ultimately the body will have excess of mutated cells causing anemia and myelofibrosis (a condition in which excess of white blood cells and scarring of bone marrow occurs). Often the gene mutation that occurs during this disorder is called as JAK2.


Who are at risk?


Certain factors like old age, history of blood cell disorder can cause myelofibrosis. People with blood cell problems like polycythemia vera or thrombocythemia would have increased chance for getting this disorder. Prolonged exposure to certain chemicals like benzene or toluene will increase the risk of myelofibrosis. People who are exposed to radiation will have more chance for myelofibrosis than others.


Complications :


Sometimes myelofibrosis will cause increased blood pressure called portal hypertension on the portal veins. This will force more blood into the small veins present in stomach causing rupture and internal bleeding. This can cause enlargement of spleen with severe abdominal pain.


When the blood cells grow outside the bone marrow it can create tumors in other areas leading to problems in gastrointestinal tract. Myelofibrosis can make the body prone to infection by increasing the number of mutated white blood cells. This in turn will make the body susceptible to infection. As the number of white blood cells outgrows the number of platelets it affects the normal functions of platelets causing heavy bleeding.


It causes swelling of connective tissue of bones causing hardening of bone marrow. Individuals with myelofibrosis will develop leukemia blood cancer quickly.


Diagnoses :


The doctor will physically examine the body checking the lymph nodes, abdomen and spleen area. He would request for blood culture, ultrasound scan and bone marrow biopsy for detecting the intensity of bone marrow disorders. Lastly he may send the blood sample for gene testing for confirming the gene mutation.


Treatment :


For mild symptoms or without any visible sign of myelofibrosis no treatment is required. Your doctor will observe the health condition closely looking for progression of any disease.


Ruxolitinib in the brand name of Jakafi is prescribed for treating this disease and setting right the symptoms of gene mutation. This drug has been approved recently by the FDA which arrests the mechanism of mutated gene of JAK and protects the healthy cells. The medicine can lower the levels of blood platelets causing easy bleeding and bruising. For people who have got anemia due to myelofibrosis blood transfusion can be done for increasing the red blood cells.


Some people resort to taking androgen therapy by injecting the male hormone which in turn produces increased red blood cells for curing anemia. Thalamid and Reylimid are prescribed for treating enlarged spleen.


In case the spleen becomes enlarged profusely then your doctor may remove it using splenectomy (surgical removal) or give chemotherapy for reducing pain and other symptoms. He would also suggest radiation therapy for some people for killing the mutated cells. Stem cell transplant is an alternate treatment for myelofibrosis in which new stem cells of the donor is injected into the patient’s body. But this procedure has some side effects like organ damage, cataract formation and cancer.


Coping With Myelofibrosis :


Individuals affected with this disorder should realize that is a rare disease and collect as much information as you can from your doctor. You can join the support group for sharing your agony with similar people. Go for regular checkups with your doctor and keep yourself updated with bone marrow examination. Engage in lot of physical activities and find relaxation through regular yoga and meditation. You can also approach a counselor for coping with the stress involved with this disease.


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