Locations Main Campus: Chesterfield, MO 63017   |   Locations
314-434-1500 314-434-1500   |   Contact Us

Multimedia Encyclopedia


 
E-mail Form
Email Results

 
 
Print-Friendly
Bookmarks
bookmarks-menu

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.

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:

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

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 unknown. There are no known risk factors. When it occurs, it often develops slowly in people over age 50.

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:

  • Abdominal fullness, pain, or feeling full before finishing a meal (because of an enlarged spleen)
  • Bone pain
  • Easy bleeding, bruising
  • Fatigue
  • Increased likelihood of getting an infection
  • Pale skin
  • Shortness of breath with exercise

 

Exams and Tests

 

The health care provider will perform a physical exam and ask about the symptoms.

Tests that may be done include:

  • Complete blood count (CBC) with blood smear to check different types of blood cells
  • Measuring tissue damage (LDH enzyme level)
  • Genetic testing
  • Bone marrow biopsy to diagnose the condition and to check for bone marrow cancers

 

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:

  • Blood transfusions and medicines to correct anemia
  • Radiation and chemotherapy
  • Medicines to target a genetic mutation linked to this disease, if present
  • Removal of the spleen (splenectomy) if swelling causes symptoms, or to help with anemia

 

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.

Survival of people with primary myelofibrosis is about 5 years. But some people survive for decades.

 

Possible Complications

 

Complications may include:

  • Development of acute myelogenous leukemia
  • Infections
  • Bleeding
  • Blood clots
  • Liver failure

 

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.

 

 

References

Mascarenhas J, Najfeld V, Kremyanskaya M, Hoffman R. Primary myelofibrosis. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 69.

Tefferi A. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 166.

 

        Tests for Myelofibrosis

         

           

          Review Date: 5/20/2016

          Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

          The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
          adam.com

           
           
           

           

           

          A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.



          Content is best viewed in IE9 or above, Firefox and Google Chrome browser.