Idiopathic myelofibrosis is a rare and serious disease of bone marrow. This condition disrupts the normal production of blood cells. The build-up of scar tissue inside the marrow cavity due to uncontrolled growth of precursors of blood cells results in this condition. The scarring of the bone marrow tissue reduces its functionality resulting in production of fewer red blood cells, too many white blood cells, and platelet counts may be increased or decreased. Fibrous tumors may also develop on the outer side of the bone marrow. Idiopathic myelofibrosis is also known as agnogenic myeloid metaplasia, aleukemic megakaryocytic myelosis, chronic myelosclerosis, and leukoerythroblastosis.
Statistics show that the annual incidence of myelofibrosis was 0.3 to 1.5 cases per 100,000 persons. Most often this condition goes unnoticed as it is less severe or asymptomatic.
Idiopathic myelofibrosis is a chronic condition which means it develops in an extended period. Symptoms also appear slowly over a prolonged period. More than half of the patients do not show any symptoms. Regular general health check-up reports might leave a clue about this condition. One such clue is an enlarged spleen.
Some of the other symptoms associated with myelofibrosis include those of anemia such as fatigue, weight loss, paleness, fever, sweating, heart palpitations, and shortness of breath. Other symptoms include itchiness, feeling full after eating a small amount of food, stomach pain or discomfort, pain in the left shoulder or upper left a portion of the body, unexpected bleeding and bone pain, especially in the legs.
Idiopathic myelofibrosis usually occurs when there is a genetic mutation in the blood stem cells or blood cell precursors. The gene mutation which results in myelofibrosis is referred to as JAK2 and other gene mutations also may be associated with this condition. Researches still say that the exact cause for the genetic mutation in blood stem cells is unclear. However, those who are exposed to benzene or high doses of radiation have the risk of developing this idiopathic myelofibrosis.
Factors which may increase your risk of Idiopathic myelofibrosis are:
The myelofibrosis treatment plan is very much customized. The main goal of treatment for idiopathic myelofibrosis is to relieve symptoms and lower the risk of complications. If you are asymptomatic, then you may not need any treatment for years.
MedicationsDrugs are used to improve the count of blood cells or relieve the symptoms. Patients with idiopathic myelofibrosis may be treated with different drugs such as ruxolitinib, hydroxyurea, interferon alfa, danazol, prednisone, anagrelide, lenalidomide, and bisphosphanate.
Supportive therapyIf you are diagnosed with anemia, supplements such as iron and folate are recommended. If the case of severe anemia, blood transfusions are recommended. Bisphosphonates can help to stop the breaking down of bone as they contribute to strengthening your bones.
Radiation therapyThe cancer cells are destroyed with the help of high-energy rays or particles and the therapy which uses these high-energy rays is known as radiation therapy. External beam radiation therapy can be used to treat an enlarged spleen, bone pain or tumors that develop outside of the bone marrow.
SurgerySplenectomy a surgical technique to remove spleen is usually performed to remove an enlarged spleen if it is causing pain. Myeolofibrosis splenectomy is also done to return blood cell counts to normal.
Stem cell transplantYounger people diagnosed with advanced idiopathic myelofibrosis are usually recommended with a stem cell transplant.
There is no specific or particular screening test to detect idiopathic myelofibrosis. However, routine blood tests are used as a screen to check the RBC, WBC, as well as the platelet counts. The results from these tests can signal for further more invasive testing, such as a bone marrow biopsy. During your routine check-ups, your doctor may ask about any general symptoms you have and based on those reports does a physical exam to check if your spleen or liver is enlarged or order other tests.
The tests used to diagnose leukemia are also used to diagnose idiopathic myelofibrosis as well. These tests include:
