Definition
Primary myelofibrosis is a disorder of the bone marrow, in which the marrow is replaced by fibrous (scar) tissue.
Symptoms
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Definition
Primary myelofibrosis is a disorder of the bone marrow, in which the marrow is replaced by fibrous (scar) tissue.
Causes
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.
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.
Treatment
There is no specific treatment for primary myelofibrosis. Blood transfusions are given to correct anemia. Recombinant erythropoietin or androgens may stimulate red blood cell production and may be beneficial. A splenectomy (removal of the spleen) may help if the enlarged size of the spleen causes symptoms. Radiation and chemotherapy may also be used. In young people, bone marrow transplants appear to improve the outlook.
Other Names
Myelofibrosis; Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia
Outlook (Prognosis)
The average survival of people with primary myelofibrosis is about 5 years. However, many people survive for decades. In the end stages, myelofibrosis is a wasting, debilitating illness.
Prevention
There is no known prevention.
References
Hoffman R, Benz Jr. EJ, Shattil SJ, et al. Hematology: Basic Principles and Practice . 4th ed. Philladelphia, Pa: Churchill Livingston; 2005.
McPherson RA and Pincus MR. Henry's Clinical Diagnosis and Management by Laboratory Methods. 21st ed. Philadelphia, Pa: WB Saunders; 2007:561-62.