Main Campus: Chesterfield, MO 63017 | 
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.
Anemia
Anemia is a condition in which the body does not have enough healthy red blood cells. Red blood cells provide oxygen to body tissues. Different type...
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.
Leukemia
Leukemia is a type of blood cancer that begins in the bone marrow. Bone marrow is the soft tissue in the center of the bones, where blood cells are ...
Symptoms
Symptoms may include any of the following:
-
Abdominal fullness
, pain, or feeling full before finishing a meal (because of an enlarged spleen)
Abdominal fullness
Gas is air in the intestine that is passed through the rectum. Air that moves from the digestive tract through the mouth is called belching. Gas is ...
Read Article Now Book Mark Article -
Bone pain
Bone pain
Bone pain or tenderness is aching or other discomfort in one or more bones.
Read Article Now Book Mark Article -
Easy bleeding,
bruising
Bruising
Bleeding into the skin can occur from broken blood vessels that form tiny red dots (called petechiae). Blood also can collect under the tissue in la...
Read Article Now Book Mark Article - Fatigue
- Increased likelihood of getting an infection
-
Pale skin
Pale skin
Paleness is an abnormal loss of color from normal skin or mucous membranes.
Read Article Now Book Mark Article -
Shortness of breath
with exercise
Shortness of breath
Breathing difficulty may involve:Difficult breathingUncomfortable breathingFeeling like you are not getting enough air
Read Article Now Book Mark Article
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
Complete blood count
A complete blood count (CBC) test measures the following:The number of red blood cells (RBC count)The number of white blood cells (WBC count)The tota...
Read Article Now Book Mark Article -
Measuring tissue damage (
LDH enzyme level
)
LDH enzyme level
The lactate dehydrogenase (LDH) isoenzyme test checks how much of the different types of LDH are in the blood.
Read Article Now Book Mark Article - Genetic testing
-
Bone marrow biopsy
to diagnose the condition and to check for bone marrow cancers
Bone marrow biopsy
A bone marrow biopsy is the removal of marrow from inside bone. Bone marrow is the soft tissue inside bones that helps form blood cells. It is foun...
Read Article Now Book Mark Article
Treatment
Bone marrow or stem cell transplant may improve symptoms, and may cure the disease. This treatment is usually considered for younger people.
Bone marrow
A bone marrow transplant is a procedure to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Bone marrow is the soft, fat...
Other treatment may involve:
- Blood transfusions and medicines to correct anemia
-
Radiation and
chemotherapy
Chemotherapy
The term chemotherapy is used to describe cancer-killing drugs. Chemotherapy may be used to:Cure the cancerShrink the cancerPrevent the cancer from ...
Read Article Now Book Mark Article - 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
Splenectomy
Spleen removal is surgery to remove a diseased or damaged spleen. This surgery is called splenectomy. The spleen is in the upper part of the belly, ...
Read Article Now Book Mark Article
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
Acute myelogenous leukemia
Acute myeloid leukemia (AML) is cancer that starts inside bone marrow. This is the soft tissue in the center of bones that helps form all blood cell...
Read Article Now Book Mark Article - 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.
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.
