Idiopathic myelofibrosis

Idiopathic myelofibrosis (IM) is a rare, chronic blood disorder characterized by the replacement of the bone marrow with fibrous tissue. This condition is also known as primary myelofibrosis or agnogenic myeloid metaplasia.

Symptoms

The symptoms of idiopathic myelofibrosis can vary greatly from person to person. Some people may have no symptoms at all, while others may experience severe symptoms. Common symptoms include:

• Fatigue
• Shortness of breath
• Weakness
• Pain in the upper left part of the abdomen
• Fever
• Night sweats
• Weight loss
• Easy bruising or bleeding
• Bone pain

Causes

The exact cause of idiopathic myelofibrosis is unknown. However, it is believed to be related to mutations in certain genes, including the JAK2 gene. These mutations are not inherited, but occur during a person's lifetime.

Diagnosis

Diagnosis of idiopathic myelofibrosis is often made based on a person's symptoms, a physical examination, and the results of certain laboratory tests. These tests may include:

• Blood tests
• Bone marrow biopsy
• Genetic testing

Treatment

There is currently no cure for idiopathic myelofibrosis. Treatment is aimed at managing symptoms and improving quality of life. Treatment options may include:

• Medication
• Blood transfusions
• Stem cell transplant

Prognosis

The prognosis for people with idiopathic myelofibrosis varies. Some people may live for many years with the disease, while others may have a more aggressive form of the disease that progresses quickly.

See also

• Myeloproliferative neoplasms
• Polycythemia vera
• Essential thrombocythemia

Idiopathic myelofibrosis Resources
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