What is the definition of Myelofibrosis?

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

What are the alternative names for Myelofibrosis?

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

What are the causes for Myelofibrosis?

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 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.

What are the symptoms for Myelofibrosis?

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
  • Weight loss
  • Night sweats
  • Low grade fever
  • Enlarged liver
  • Dry cough
  • Itchy skin

What are the current treatments for Myelofibrosis?

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
  • Targeted medicines
  • Removal of the spleen (splenectomy) if swelling causes symptoms, or to help with anemia

What is the outlook (prognosis) for Myelofibrosis?

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.

What are the possible complications for Myelofibrosis?

Complications may include:

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

When should I contact a medical professional for Myelofibrosis?

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

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.

Long NM, Kavanagh EC. Myelofibrosis. In: Pope TL, Bloem HL, Beltran J, Morrison WB, Wilson DJ, eds. Musculoskeletal Imaging. 2nd ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 76.

Mascarenhas J, Najfeld V, Kremyanskaya M, Keyzner A, Salama ME, Hoffman R. Primary myelofibrosis. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 70.

  • Condition: Myelofibrosis (MF)
  • Journal: Hematology/oncology clinics of North America
  • Treatment Used: Stem Cell Therapy
  • Number of Patients: 0
  • Published —
This article discusses the use of stem cell therapy in patients with myelofibrosis (MF).