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Myelofibrosis

Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia; Primary myelofibrosis; Secondary myelofibrosis; Bone marrow - myelofibrosis

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

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Causes

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

When the bone marrow is scarred, it cannot make enough blood cells. Anemia, bleeding problems, and a higher risk for infections may occur.

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

The cause of myelofibrosis is often unknown. There are no known risk factors. When it occurs, it often develops slowly in people over age 50. Women and men are equally affected. There is an increased occurrence of this condition in Ashkenazi Jews.

Blood and bone marrow cancers, such as myelodysplastic syndrome, leukemia, and lymphoma, may also cause bone marrow scarring. This is called secondary myelofibrosis.

Symptoms

Symptoms may include any of the following:

Exams and Tests

Your health care provider will perform a physical exam and ask about your symptoms.

Tests that may be done include:

Treatment

Bone marrow or stem cell transplant may improve symptoms, and may cure the disease. This treatment is usually considered for younger people.

Other treatment may involve:

Outlook (Prognosis)

As the disease worsens, the bone marrow slowly stops working. Low platelet count leads to easy bleeding. Spleen swelling may get worse along with anemia.

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

Possible Complications

Complications may include:

When to Contact a Medical Professional

Make an appointment with your provider if you have symptoms of this disorder. Seek medical care right away for uncontrolled bleeding, shortness of breath, or jaundice (yellow skin and whites of the eyes) that gets worse.

Related Information

Anemia
Platelet count
Splenomegaly
Acute myeloid leukemia - adult

References

Gotlib J. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 157.

Venugopal S, Najfeld V, Keyzner A, Jamal SME, Ronald H, Mascarenhas J. Primary myelofibrosis and chronic neutrophilic leukemia. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 70.

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Review Date: 6/17/2024  

Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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