Myelofibrosis

Myelofibrosis is a haematological disorder where there is the replacement of bone marrow with collagenous connective tissue and progressive fibrosis. It is also classified as a myeloproliferative disorder. It is characterised by:

It usually affects the middle-aged to elderly (mean age 60 years6). The estimated prevalence is at ~1:100,000.

Non-neoplastic fibroblasts produce collagen, which replaces normal bone marrow elements. This bone marrow fibrosis is a result of an inappropriate release of PDGF and TGF-ß from neoplastic megakaryocytes 8.

Subtypes

It can be broadly classified into:

Most radiological features are a result of EMH and seen in many systems.

General
Musculoskeletal
  • osteosclerosis
    • tends to be diffuse and there is a lack of architectural distortion
    • frequent sites include
      • axial skeleton
      • ribs
      • proximal humerus and femur
    • bone scan may give "superscan" appearance
Abdominal
Cardiovascular
  • may show evidence of congestive cardiac failure due to anaemia 3

Prognosis is poor, with slow progression and death usually within 2-3 years. It can also transform into acute myeloid leukaemia (AML) in a small number of patients 10.

Complications

General differential considerations include:

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Article Information

rID: 10893
Section: Pathology
Synonyms or Alternate Spellings:
  • Myeloid metaplasia

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    Case 1: with widespread decreased marrow signal
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    Case 6: with hepatosplenomegaly
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