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Alternative namesPrimary myelofibrosis is a disorder of the bone marrow in which the marrow is replaced by fibrous (scar) tissue.
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.
Signs and testsPhysical examination shows an enlarged spleen. Later in the disease, it may also show an enlarged liver .
TreatmentThere 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.
Expectations (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.
Calling your health care providerCall for an appointment with your health care provider if symptoms of this disorder develop. Uncontrolled bleeding, shortness of breath, jaundice , and progressive confusion are symptoms that indicate a need for urgent or emergency care.
PreventionThere is no known prevention.
Update Date: 7/31/2003Corey Cutler, M.D., M.P.H., F.R.C.P.C., Department of Medical Oncology, Dana-Farber Cancer Institute; Instructor of Medicine, Harvard University, Boston, MA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT