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Primary myelofibrosis Causes and Risks:
In primary
myelofibrosis, blood formation occurs 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 persons 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 is progressive without a cure. The incidence is 2 out of 100,000 people.
Prevention:
There is no known prevention.
Symptoms:
Signs and Tests:
Physical examination shows an enlarged spleen . Later in the disease it may also show an enlarged liver .
This disease may also alter the results of the following tests:
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. Splenectomy (removal of the spleen) may be indicated where splenic enlargement causes symptoms related to its size. Radiation and chemotherapy may also be used.
Prognosis:
The median survival of people with primary myelofibrosis is about 5 years. However, many people survive for decades. End-stage disease is a wasting illness with debility.
Complications:
Call Your Healthcare Provider:
Call 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.
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