Agnogenic myeloid metaplasia
    
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Agnogenic myeloid metaplasia

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

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

Exams 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.

Outlook (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.

Possible Complications

When to Contact a Medical Professional

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.

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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