Myelofibrosis is a bone marrow disorder that leads to a disruption of the body’s ability to produce normal healthy blood cells. A rare form of chronic leukemia, the disease often worsens over time and may cause some patients to develop more serious types of leukemia. Conversely, other people have myelofibrosis and go for years without noticing any symptoms.
Myelofibrosis occurs when a genetic mutation causes damage to a single blood-forming stem cell. As this cell grows and replicates, the mutation is passed on to the new cells. In time, the abnormal cells begin to affect the process of blood cell production and overtake the healthy cells. The key feature distinguishing myelofibrosis from other blood cancers is the overproduction of megakaryocytes, or the platelet-forming cells. Too many platelets are released, creating substantial scar tissue within the bone marrow. The exact reason for the mutation that causes the condition is not known.
Myelofibrosis is slow-progressing disease. While symptoms may not be noticed in the beginning, they will begin to develop as blood cell disruption increases.
Patients who are not experiencing symptoms may not need immediate medical care. A doctor is more likely to recommend regular examinations to monitor the condition, and treatment may not begin unless complications arise.
When therapy is needed, the focus is on relieving the symptoms. Depending on how the disorder is progressing, patients might require blood transfusions, various medications, or androgen therapy. Those with an enlarged spleen may undergo chemotherapy, radiation therapy, or a splenectomy. The only way to cure myelofibrosis is with a stem cell transplant, but there are significant risks associated with the procedure.