Primary myelofibrosis ( myelosclerosis or myeloid metaplasia )

A 43 year old man with history of primary myelofibrosis attended the computed tomography department

Myelofibrosis, one of the myeloproliferative diseases, is the gradual replacement of the bone marrow by connective tissue. The typical patient is a middle-aged or elderly adult and 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  and 5. increase fibrotic process in the bone marrow. The usual presenting complaints are fatigue and abdominal fullness due to hepatosplenomegaly. The radiological studies demonstrate increased density of the bones due to new bone deposition on the trabeculae and to the endosteal cortical thickening with loss of the normal cortico-medullary distinction. Differentiation must be made from other conditions causing a generalised increase in bone density, including congenital skerosing dysplasias ( osteopetrosis ), mastocytosis, and sclerosing metastases, particularly prostatic

References

1)     David Sutton, Textbook of radiology and imaging, Vol 2, seventh edition

2)     Myelofibrosis, Wikipedia, the free encyclopedia

3)      J Martin Johnston, MD, Myelofibrosis, e-medicine