Health Encyclopedia

Primary myelofibrosis

Definition

Primary myelofibrosis is a disorder of the bone marrow, in which the marrow is replaced by fibrous (scar) tissue.

Alternative Names

Myelofibrosis; Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia

Causes, incidence, and risk factors

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.

Symptoms

Abdominal fullness related to an enlarged spleen
Bone pain
Bruising
Easy bleeding
Fatigue
Increased susceptibility to infection
Pallor
Shortness of breath while doing physical work

Signs and tests

Physical examination shows an enlarged spleen. Later in the disease, it may also show an enlarged liver.

An examination of the blood shows teardrop-shaped red blood cells.
Bone marrow biopsy may be performed to rule out other causes of the symptoms.
CBC (complete blood count) shows a low red blood cell count.
The platelet count is variable.
The white blood cell count is variable.

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.

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.

Complications

Liver failure
Acute myelogenous leukemia

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 progressive confusion are symptoms that indicate a need for urgent or emergency care.

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.

Review Date:2/26/2007
Reviewed By:William Matsui, MD, Assistant Professor of Oncology, Division of Hematologic Malignancies, The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD. Review provided by VeriMed Healthcare Network.

ADAM Quality A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The Agency for Health Care Administration (Agency) and this website do not claim the information on, or referred to by, this site is error free. This site may include links to websites of other government agencies or private groups. Our Agency and this website do not control such sites and are not responsible for their content. Reference to or links to any other group, product, service, or information does not mean our Agency or this website approves of that group, product, service, or information.

Additionally, while health information provided through this website may be a valuable resource for the public, it is not designed to offer medical advice. Talk with your doctor about medical care questions you may have.