Myelofibrosis is a condition that develops when the bone marrow stem cells that produce blood become replaced by fibrous tissue. This internal scarring of the bone marrow and the changes in these cells can develop into acute myeloid leukemia, also known as acute myelogenous leukemia, a deadly and fast-growing form of leukemia.
Causes and Risk Factors
Myelofibrosis occurs most often in adults over 50 and grows slowly. According to MayoClinic.com, a susceptibility to the disease can be inherited and has been linked with two specific genes called JAK2 and MPL. In some cases, exposure to certain toxic chemicals can also raise the risk of developing myelofibrosis and its resultant leukemia. Chemicals linked to this disorder include toluene, benzene and the medical contrast material called Thorotrast, which was used through the 1940s.
Symptoms
According to the Leukemia and Lymphoma Society, about 25 percent of people with myelofibrosis show no symptoms at all. Others may experience weight loss, fatigue, shortness of breath, night sweats, weakness, bone pain or an enlarged spleen. Anemia is a common symptom of myelofibrosis as the red blood cells decrease in number and become unable to carry oxygen efficiently. If myelofibrosis develops into acute myeloid leukemia, additional symptoms may develop, including bleeding from the nose or gums, skin rash, paleness and fever.
Tests
If a doctor suspects a patient of having myelofibrosis, he will likely order a CBC, complete blood count, to look at the quantity and shape of red blood cells, which appear teardrop-shaped in people with this disorder. If a physical exam indicates enlarged organs, further tests such as MRI and ultrasound may be used to determine the degree of swelling and specific organs affected. A biopsy of bone marrow tissue may be necessary to finalize the diagnosis and determine whether the disease has progressed to leukemia.
Treatment
For both myelofibrosis and myeloid leukemia, treatment mainly involves reducing symptoms. Blood transfusions and medicines to increase blood cell production may help keep red blood cell numbers within a normal range. In advanced cases, a doctor may recommend radiation, chemotherapy or removal of the spleen. A bone marrow or stem cell transplant is the only treatment that holds the possibility of completely curing the disease.
Prognosis
The average person diagnosed with myelofibrosis tends to live about five years past the initial diagnosis, although some patients can survive for a decade or more, explains Medline Plus. People without a genetic component to the disease tend to do better. Some patients go into remission, with symptoms disappearing for years at a time.
