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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.

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.

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