Idiopathic myelofibrosis is a condition where scar tissue replaces bone marrow due to a mutation of hematopoietic stem cells. Symptoms include immune system compromise, respiratory issues, and organ dysfunction. Complications can include liver failure and leukemia. Treatment depends on symptom severity and can include blood transfusions or surgery.
Idiopathic myelofibrosis is a serious medical condition that negatively affects bone marrow formation. Individuals with this condition often experience a variety of symptoms that compromise their immune system, respiratory system, and organ function. Often developing in late adulthood, treatment for idiopathic myelofibrosis depends on the presentation and severity of symptoms. Complications associated with this condition can include liver failure and leukemia.
The manifestation of idiopathic myelofibrosis presents itself with the development of scar tissue in place of bone marrow due to an acquired mutation of hematopoietic stem cells. The production of red blood cells normally occurs within the bone marrow during a process known as hematopoiesis. In the presence of this type of mutation, as occurs within hematopoietic stem cells, blood cell production becomes unbalanced and scar tissue develops as a reaction to the disruption of proper bone marrow function. While there is no known single cause for developing this type of hematopoietic stem cell mutation, it has been suggested that exposure to certain environmental toxins, such as benzene, may contribute to the onset of symptoms.
Myelofibrosis is usually detected when blood tests are administered, such as a complete blood and platelet count. A blood draw is usually used to evaluate the condition and the number of red and white blood cells, hemoglobin levels and platelet count. If abdominal distension is present, further imaging tests may be done to evaluate the condition and function of the liver. A diagnosis of idiopathic myelofibrosis is usually confirmed with the administration of a bone marrow biopsy.
As an acquired condition, idiopathic myelofibrosis generally takes years to develop before symptoms appear. Presenting in the later years of adulthood, usually in those 50 years of age or older, idiopathic myelofibrosis can induce a variety of signs and symptoms that appear and progress gradually. Often, individuals may find that they begin to bruise more easily and may feel fatigued with little or no effort. It is not uncommon for people with this condition to experience shortness of breath that occurs without an obvious trigger, bone discomfort, and night sweats.
Individuals who receive a diagnosis of idiopathic myelofibrosis can develop a variety of complications depending on the severity and progression of their condition. Because of the effect idiopathic myelofibrosis has on the immune system and waste production, some may develop an increased susceptibility to infections or secondary conditions, such as gout or cancer. Additional complications may include acute leukemia, joint inflammation, vein rupture, and portal hypertension which can compromise proper liver function.
Asymptomatic individuals, that is, those who do not experience any symptoms, often do not receive treatment until the disease progresses and symptoms begin to appear. Most symptomatic individuals receive treatment based on the type and severity of symptoms. Those who develop anemia may receive periodic blood transfusions or drug therapy to increase blood and platelet counts. In the presence of certain complications, such as an enlarged spleen, drug therapies or surgery may be performed to remove the inflamed organ to prevent further complications.
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