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...
Read Article Now Book Mark ArticleAs 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.
Myelodysplastic syndrome
Myelodysplastic syndrome is a group of disorders in which the blood cells produced in the bone marrow do not mature into healthy cells. This leaves ...
Read Article Now Book Mark ArticleLeukemia
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 ...
Read Article Now Book Mark ArticleSymptoms
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
Fatigue
Fatigue is a feeling of weariness, tiredness, or lack of energy.
Read Article Now Book Mark Article - 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 breathing Uncomfortable breathingFeeling like you are not getting enough air
Read Article Now Book Mark Article - Weight loss
Weight loss
Unexplained weight loss is a decrease in body weight, when you did not try to lose the weight on your own. Many people gain and lose weight. Uninten...
Read Article Now Book Mark Article - Night sweats
- Low grade fever
- Enlarged liver
- Dry cough
- Itchy skin
Exams and Tests
Your health care provider will perform a physical exam and ask about your 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 white blood cells (WBC count)The number of red blood cells (RBC count)The numb...
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, called isoenzymes, are present in the blood.
Read Article Now Book Mark Article - Molecular 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 one of your bones. Bone marrow is the soft tissue inside bones that helps form blood cells...
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 or stem cell transplant
A bone marrow transplant is a procedure to replace damaged or diseased bone marrow with healthy bone marrow stem cells. Bone marrow is the soft, fatt...
Read Article Now Book Mark ArticleOther treatment may involve:
- Blood transfusions and medicines to correct anemia
Blood transfusions
Rheumatoid arthritis (RA) is a disease that leads to inflammation of the joints and surrounding tissues. It is a long-term disease. It can also aff...
Read Article Now Book Mark Article - Radiation and chemotherapy
Radiation
Radiation therapy uses high-powered radiation (such as x-rays or gamma rays), particles, or radioactive seeds to kill cancer cells.
Read Article Now Book Mark ArticleChemotherapy
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 - Targeted medicines
Targeted medicines
Targeted therapy uses medicines to stop cancer from growing and spreading. It does this with less harm to normal cells than other treatments. Stand...
Read Article Now Book Mark Article -
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.
The average 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.
Jaundice
Jaundice is a yellow color of the skin, mucus membranes, or eyes. The yellow coloring comes from bilirubin, a byproduct of old red blood cells. Jau...
Read Article Now Book Mark ArticleReviewed 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.
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.