Myelofibrosis

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Myelofibrosis
Classification & external resources

ICD-10	C94.5, D47.1
ICD-9	289.89
ICD-O:	9932, 9961
DiseasesDB	8616
MeSH	C15.378.190.636.636

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. The disease progresses very slowly, however, and treated patients can live for five, ten, and even eighteen years after diagnosis. Since the typical patient is older, and 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--the procedure 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.

[edit] Further reading

For more information, see:

• CMPD Education Foundation