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Primary myelofibrosis - Treatment

Alternative Names

Myelofibrosis; Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia

Treatment:

There is no specific treatment for primary myelofibrosis. The goal of treatment is to relieve symptoms. Treatment may involve:

Blood transfusions to correct anemia
Radiation and chemotherapy
Medicines called recombinant erythropoietin or androgens to help stimulate red blood cell production
Splenectomy (removal of the spleen) if swelling causes symptoms

In young people, bone marrow transplants appear to improve the outlook.

Expectations (prognosis):

This disorder causes slowly worsening bone marrow failure with severe anemia. Low platelet count leads to easy bleeding, and spleen swelling may slowly get worse.

The average survival of people with primary myelofibrosis is about 5 years. However, many people survive for decades.

Complications:

Acute myelogenous leukemia
Liver failure

Calling your health care provider:

Call for an appointment with your health care provider if symptoms of this disorder develop. Uncontrolled bleeding, shortness of breath, jaundice, and confusion that gets worse require urgent or emergency care.

Reviewed last on: 7/11/2008
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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.

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