What is myelofibrosis? Myelofibrosis is a chronic disease that may result in the scarring of bone marrow and the production of abnormal red blood cells, white blood cells, and platelets. Symptoms may include anemia, frequent infections, fatigue or tiredness, bleeding easily or bruising easily, weight loss with an inability to gain weight, enlarged liver and spleen (hepatosplenomegaly), fever, chills, and night sweats.
What causes myelofibrosis?
It is believed that myelofibrosis results from a genetic mutation. How this mutation develops is poorly understood at this time. The mutation appears to be acquired rather than inherited from one’s parents. Myelofibrosis can affect individuals of any age but is most often diagnosed in individuals between the ages of 40 and 70.
Myelofibrosis is thought to be a clonal myeloproliferative neoplasm, which means that it is one type of blood cancer that originates from cells in an immature state (blast cells). This form of cancer can involve any cell or group of cells in the bone marrow and may affect them by interfering with their normal function. In general, myelofibrosis is rare cancer that mainly affects older adults.
How common is myelofibrosis?
Myelofibrosis and its related diseases (e.g., polycythemia vera and essential thrombocytosis) represent about 1% of all myeloproliferative neoplasms and appear to be increasing in incidence over time. About 40,000 individuals in the U.S. are believed to have this disorder, although few cases may ultimately come to medical attention (i.e., may not present clinically). It is more frequently diagnosed in men than women; however, no clear reason for this difference has been established.
What are the symptoms of myelofibrosis?
Symptoms may include anemia, frequent infections, fatigue or tiredness, bleeding easily or bruising easily, enlarged liver and spleen (hepatosplenomegaly), fever, chills, and night sweats. Weight loss with an inability to gain weight is also commonly observed in individuals with myelofibrosis. It has been suggested that individuals with this disease have a poor prognosis if they present with symptomatic splenomegaly or hepatomegaly at diagnosis or develop these conditions within one year of their diagnosis. Other common complications may include high blood pressure in the lungs (pulmonary hypertension), portal hypertension (which can cause fluid accumulation in the abdomen, known as ascites), irregular heartbeats (arrhythmias), and an increased risk of developing blood clots in the veins.
What are the standard treatments for myelofibrosis?
What is myelofibrosis, treatment options include medication to control symptoms, blood transfusions, splenectomy (i.e., surgical removal of the spleen), chemotherapy or other medications, stem cell transplantation, radiation therapy to treat active bone marrow cancer, and supportive care measures. Disease-related complications may also be treated with surgery if necessary. Research suggests that individuals diagnosed with this disease have a reduced life expectancy compared to the general population; however, survival has improved over time due to progress in treatment technologies.
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