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Myelofibrosis

 

Definition

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

Alternative Names

Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia; Primary myelofibrosis; Secondary myelofibrosis

Causes

Bone marrow is the soft, fatty tissue inside your bones. Stem cells are immature cells in the bone marrow that give rise to all of your blood cells. Your blood is made of:

  • Red blood cells (which carry oxygen to your tissues)
  • White blood cells (which fight infection)
  • Platelets (which help your blood clot)

Scarring of the bone marrow means the marrow is not able to make enough blood cells. Anemia, bleeding problems, and a higher risk of infections may occur.

As a result, the liver and spleen try to make some of these blood cells. This causes these organs to swell, which is called extramedullary hematopoiesis.

The cause of myelofibrosis is unknown. There are no known risk factors. The disorder usually develops slowly in people over age 50.

Diseases such as leukemia and lymphoma may also cause bone marrow scarring. This is called secondary myelofibrosis.

Symptoms
Signs and tests

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

Tests that may be done include:

  • CBC (complete blood count)

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.

Support Groups

Expectations (prognosis)

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

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

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, or jaundice that gets worse need urgent or emergency care.

Complications
Treatments

There is no specific treatment for myelofibrosis. Treatment depends on the symptoms and degree of the low blood counts.

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, or to help with anemia

In young people, bone marrow or stem cell transplants appear to improve the outlook, and may cure the disease.

Prevention

There is no known prevention.

References

Tefferi A. Myeloproliferative disorders: essential thrombocythemia and primary myelofibrosis. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 177.


Review Date: 6/2/2010
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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