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Chronic Myeloproliferative Disorders Treatment

Primary Myelofibrosis

Primary myelofibrosis is a disease in which abnormal blood cells and fibers build up inside the bone marrow.

The bone marrow is made of tissues that make blood cells (red blood cells, white blood cells, and platelets) and a web of fibers that support the blood-forming tissues. In primary myelofibrosis (also called chronic idiopathic myelofibrosis), large numbers of blood stem cells develop into blood cells that do not mature properly (blasts). The web of fibers inside the bone marrow also becomes very thick (like scar tissue) and slows the blood-forming tissue’s ability to make blood cells. This causes the blood-forming tissues to make fewer and fewer blood cells. In order to make up for the low number of blood cells made in the bone marrow, the liver and spleen begin to make the blood cells.

Possible signs of primary myelofibrosis include pain below the ribs on the left side and feeling very tired.

Primary myelofibrosis often does not cause early symptoms. It is sometimes found during a routine blood test. The following symptoms may be caused by primary myelofibrosis or by other conditions. A doctor should be consulted if any of these problems occur:

A special blood test is used to diagnose primary myelofibrosis.

In addition to a complete blood count, bone marrow aspiration and biopsy, and cytogenetic analysis, a peripheral blood smear is used to diagnose primary myelofibrosis. A peripheral blood smear is a procedure in which a sample of blood is checked for tear drop-shaped red blood cells, the number and kinds of white blood cells, the number of platelets, and the presence of blast cells.

Certain factors affect prognosis (chance of recovery) and treatment options for primary myelofibrosis.

Prognosis (chance of recovery) depends on the following: