Myelofibrosis—also called agnogenic myeloid metaplasia (AMM), myelosclerosis, chronic idiopathic myelofibrosis, idiopathic myelofibrosis, myelosclerosis with myeloid metaplasia, and primary myelofibrosis—is a form of cancer that arises in the bone marrow.

A progressive, chronic disease in which the bone marrow is replaced by fibrous tissue and blood is made in organs such as the liver and the spleen, instead of in the bone marrow. This disease is marked by an enlarged spleen and progressive anemia. Also called chronic idiopathic myelofibrosis, idiopathic myelofibrosis, myelosclerosis with myeloid metaplasia, and primary myelofibrosis.

Causes

Myelofibrosis occurs when the bone marrow produces too much collagen or fibrous tissue. As a result, fewer blood-producing cells are created, and they can be destroyed more rapidly, which leads to anemia (low levels of red blood cells), low platelet count, and an increased risk of developing infections. This condition can occur by itself or in combination with other myeloproliferative disorders, such as essential thrombocytosis or polycythemia.

Symptoms

Myelofibrosis symptoms are somewhat similar to chronic myelogenous leukemia (CML), but patients lack the genetic defect known to cause CML.

Patients tend to be over 60 years old, and experience an enlarged spleen and anemia as the bone marrow stops making blood and other organs like the liver and spleen attempt to take over blood production.

Other symptoms may include:

• Fatigue
• A general feeling of discomfort
• Difficulty breathing
• Weight loss
• Fever and night sweats
• Anemia
• Abnormal bleeding

Patients with myelofibrosis have an increased risk of bleeding. They are more susceptible to developing infections compared to healthy individuals. In addition, patients have an increased risk of developing an enlarged spleen. In extreme cases, the spleen may rupture.

Treatment of Myelofibrosis

Bone marrow transplantation

Blood and marrow transplantation is one of the specialty therapies available for the treatment of myelofibrosis. The BMT Program at Stanford has been very successful with a history of limited morbidity rates and acute mortality that is well below most published reports.

A bone marrow transplant, also called a stem cell transplant, is a procedure to replace your diseased bone marrow using healthy blood stem cells. For myelofibrosis, the procedure uses stem cells from a donor (allogeneic stem cell transplant).

This treatment has the potential to cure myelofibrosis, but it also carries a high risk of life-threatening side effects, including a risk that the new stem cells will react against your body's healthy tissues (graft-versus-host disease).

Many people with myelofibrosis, because of age, stability of the disease or other health problems, don't qualify for this treatment.

Prior to a bone marrow transplant, you receive chemotherapy or radiation therapy to destroy your diseased bone marrow. Then you receive infusions of stem cells from a compatible donor.

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