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Primary myelofibrosis
Definition:
Primary myelofibrosis is a disorder of the bone marrow in which the marrow is replaced by fibrous (scar) tissue. Alternative Names:
Myelofibrosis; Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia Text Continues Below
Causes, incidence, and risk factors:
In primary myelofibrosis, a progressive scarring (fibrosis) of the bone marrow occurs. This causes blood formation to take place in sites other than the bone marrow, such as the liver and spleen, causing enlargement of these organs. The cause is unknown. 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. The disease gets progressively worse in most cases. Risk factors are unknown.
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