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Alternative NamesMyelofibrosis; Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia
Definition Return to top
Primary myelofibrosis is a disorder of the bone marrow, in which the marrow is replaced by fibrous (scar) tissue.
Causes Return to top
The cause of primary myelofibrosis is unknown. Scarring of the bone marrow causes blood to form in abnormal sites, such as the liver and spleen. This causes swelling of these organs.
There are no known risk factors.
The disorder usually develops slowly in people over age 50.
Symptoms Return to top
Exams and Tests Return to top
Physical examination shows spleen swelling. Later in the disease, it may also show an enlarged liver.
Tests that may be done include:
An examination of the blood shows teardrop-shaped red blood cells. Bone marrow biopsy may be done to rule out other causes of the symptoms.
Treatment Return to top
There is no specific treatment for primary myelofibrosis. The goal of treatment is to relieve symptoms. Treatment may involve:
In young people, bone marrow transplants appear to improve the outlook.
Outlook (Prognosis) Return to top
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.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
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.
Prevention Return to top
There is no known prevention.
References Return to top
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.Update Date: 7/11/2008 Updated by: 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.