Myelofibrosis, one of the myeloproliferative diseases, is the gradual replacement of the bone marrow by connective tissue.

The replacement of the bone marrow tissue reduces the patient's ability to generate new blood cells resulting in chronic anemia. A prime feature is "extramedullary hematopoeisis", i.e. the remaining blood-forming cells migrate to other sites in the body, e.g. the liver or spleen. Patients will typically have an enlarged spleen and liver, (hepatosplenomegaly), and examination of the blood cells will show "teardrop cells".

In order to diagnose myelofibrosis, the following criteria must be fulfilled: (1) no evidence of other myeloproliferative disorder (2) no evidence of secondary bone marrow fibrosis (3) leucoerythroblastic picture on blood film (4) presence of splenomegaly (5) increase fibrotic process in the bone marrow.

Typically affecting patients more than 50 years old, it is a chronic and debilitating condition. Currently the only known cure is a bone marrow transplant. However the disease moves very slowly and patients can live for more than five or ten and even as long as eighteen more years after diagnososis, (with treatment). Since the bone marrow replacement treatment is very rigorous, painful and high risk, involving the use of chemotherapy to destroy the patient's existing marrow tissue, and the typical patient is older, the bone marrow treatment is often not used.

There are other treatments available to reduce the effects of the disease, which are repeated at regular intervals to maintain quality of life for the patient.

Further reading

For more information, see:

• Myeloproliferative Disease Support List
• Association of Cancer Online Resource (ACOR) Myeloproliferative Disorders page

Hematology