Myleofibrosis

Primary myelofibrosis, also known as Myelofibrosis; Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia, is a disorder of the bone marrow, in which the marrow is replaced by fibrous (scar) tissue (collagen).

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Causes, incidence, and risk factors

In primary myelofibrosis, a progressive scarring (fibrosis) of the bone marrow occurs. As a result, blood forms in sites other than the bone marrow, such as the liver and spleen. This causes an enlargement of these organs. The cause and risk factors are unknown. It commonly occurs in the spent phase of Polycythemia Rubra Vera, possibly in response to the medication hydroxyurea poisoning the marrow.

The disorder usually develops slowly, in people over 50 years old. It leads to progressive bone marrow failure with severe anemia. Low platelet count leads to easy bleeding, and spleen enlargement continues.

Symptoms

• Abdominal fullness related to an enlarged spleen
• Bone pain
• Bruising
• Easy bleeding
• Fatigue
• Increased susceptibility to infection
• Pallor
• Shortness of breath while doing physical work
• Uncontrollable diarrhea

Treatment

Myleofibrosis has no known cure. Lenalidomide (brand name Revlimid) and Thalidomide are drugs that can be used in its treatment, though they can cause gout and leave the patient susceptible to diseases such as pneumonia. Frequent blood transfusions may also be required.