Myelofibrosis (patient information)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief:Sabawoon Mirwais, M.B.B.S, M.D.[2] Lakshmi Gopalakrishnan, M.B.B.S. [3]

Synonyms and keywords: Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia; Primary myelofibrosis; Secondary myelofibrosis

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

• Abdominal fullness related to an enlarged spleen
• Bone pain
• Bruising
• Easy bleeding
• Fatigue
• Increased likelihood of getting an infection
• Pallor
• Shortness of breath with exercise
• Weight loss
• Loss of appetite
• Night sweats
• Fever
• Early satiety
• Bleeding
• Peripheral swelling
• Frequent infections
• Dry cough
• Skin nodules
• Itchy skin

• Bone marrow is the soft, fatty tissue inside your bones. Stem cells are immature cells in the bone marrow that give rise to all of your blood cells. Your blood is made up of:

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

• Scarring of the bone marrow means the marrow is not able to make enough blood cells. Anemia, bleeding problems, and a higher risk of infections may develop as a result.

• The liver and spleen may try to compensate and make some of these blood cells, a process called ‘extramedullary hematopoiesis’. This causes these organs to swell.

• The disorder usually develops slowly in people over age 50.

• Diseases such as leukemia and lymphoma may also cause bone marrow scarring. This is called secondary myelofibrosis.

• Physical examination shows swelling of the spleen. Later in the disease, it may also show an enlarged liver.

• Tests that may be done, include CBC (complete blood count), bone marrow biopsy, and genetic testing.

• An examination of the blood shows teardrop-shaped red blood cells. Bone marrow biopsy may be done to rule out other causes of the symptoms.

Call for an appointment with your health care provider if symptoms of this disorder develop. Uncontrolled bleeding, shortness of breath, or jaundice that gets worse, need urgent or emergency care.

• There is no specific treatment for myelofibrosis. Treatment depends on the symptoms and degree of the low blood cell counts.

• The goal of treatment is to alleviate the symptoms. Treatment may involve:

• Blood transfusions to correct anemia
• Radiation and chemotherapy
• Medications such as recombinant erythropoietin or androgens to help stimulate red blood cell production
• Splenectomy (removal of the spleen), if swelling causes symptoms, or to help with anemia

• In young people, bone marrow or stem cell transplantation can improve the outlook, and may cure the disease.

Directions to Hospitals Treating Myelofibrosis

There is no known prevention.

This disorder causes slowly worsening bone marrow failure with severe anemia. Low platelet count leads to easy bleeding. Spleen swelling may slowly get worse.

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

• Acute myeloid leukemia
• Liver failure
• Bleeding
• Thrombotic events
• Heart failure
• Splenic rupture
• Pulmonary hypertension
• Peritonitis
• Spinal cord compression
• Intestinal obstruction
• Kidney problems

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