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Causes
By Mayo Clinic staffThe process of producing blood cells in your body is called hematopoiesis. This process starts in your bone marrow with a certain type of cell called a hematopoietic stem cell. A stem cell is a primitive, undifferentiated cell that has the ability to replicate and then divide into multiple specialized cells — red blood cells, white blood cells and platelets.
These specialized blood cells have a limited life span. When they grow old, they die off naturally and are replaced by new cells, in a continuous, healthy cycle.
How myelofibrosis occurs
Myelofibrosis occurs when the genetic material in a single hematopoietic stem cell changes, or mutates. What causes this mutation is often unknown, but it's usually an acquired mutation that develops during your lifetime rather than one that's present at birth (congenital).
When the mutated cell replicates and divides, it passes along the mutation to the new cells. As more and more of these mutated cells are created, they begin to have serious effects on blood production. The end result is usually a lack of red blood cells — which causes the anemia characteristic of myelofibrosis — and an overabundance of white blood cells with varying levels of platelets.
Because of the overproduction of white blood cells, doctors refer to myelofibrosis as a myeloproliferative disease, a type of disease characterized by uncontrolled production of one or more types of blood cells. Scarring (fibrosis) within the bone marrow is thought to be a secondary reaction to the activity of the mutated cells. Your spleen and sometimes your liver may become enlarged when they trap excess white blood cells circulating through your body.
- Tefferi A. Clinical manifestations and diagnosis of primary myelofibrosis (agnogenic myeloid metaplasia). http://www.uptodate.com/home/index.html. Accessed Oct. 14, 2008.
- Tefferi A. Prognosis and treatment of primary myelofibrosis (agnogenic myeloid metaplasia). http://www.uptodate.com/home/index.html. Accessed Oct. 14, 2008.
- Tefferi A. Pathogenic mechanisms in primary myelofibrosis (anogenic myeloid metaplasia). http://www.uptodate.com/home/index.html. Accessed Oct. 14, 2008.
- Cervantes F. Modern management of myelofibrosis. British Journal of Haemotology. 2005;128(5):583-92.
- Papageorgiou SG, et al. Allogeneic stem cell transplantation as treatment for myelofibrosis. Bone Marrow Transplant. 2006;38(11):721-727.
- Idiopathic myelofibrosis. Leukemia & Lymphoma Society. http://www.leukemia-lymphoma.org/attachments/National/br_1190656475.pdf. Accessed Oct. 14, 2008.
- Chronic idiopathic myelofibrosis. National Cancer Institute. http://www.cancer.gov/cancertopics/pdq/treatment/myeloproliferative/HealthProfessional/page5. Accessed Oct. 14, 2008.
- Myelofibrosis with myeloid metaplasia. MayoClinic.org. http://www.mayoclinic.org/myelofibrosis/treatment.html. Accessed Oct. 14, 2008.
- Reduced-intensity transplants. National Marrow Donor Program. http://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Undrstnd_Treat_Opt/Lrn_BMT_Cord/R_Intensity_Tx/index.html. Accessed Oct. 14, 2008.
