Myelofibrosis is a haematological disorder where there is replacement of bone marrow with collagenous connective tissue and progressive fibrosis. It is also classified as a myeloproliferative disorder. Patients are often anaemic.
It usually affects the middle aged to elderly (mean age 60 6). The estimated prevalence is at ~ 1 : 100,000.
It can be broadly classified into
- primary myelofibrosis - e.g. agnogenic myeloid metaplasia
- secondary myelofibrosis - marrow replacement from malignancy / toxins
Most radiological features are a result of extramedullary haematopoeisis (EMH) and seen in many systems
- tends to be diffuse and there is a lack of architectural distortion
- frequent sites include
- axial skeleton (insive of pelvis and skull)
- proximal humerus and femur.
- a bone scan may give "superscan" appearance
- splenomegaly - can be massive
- evidence of portal hypertension 3
- from increased splenic blood flow
- from portal flow obstruction from sinusoidal haematopoetic proliferation
- may show evidence of congestive cardiac failure due to anaemia 3.
- gout : from hyperuricaemia due to increased haematopoeitic turnover
General differential considerations include
- 1. Bond JR. Musculoskeletal case of the day. Myelofibrosis. AJR Am J Roentgenol. 1993;160 (6): 1330. AJR Am J Roentgenol (citation) - Pubmed citation
- 2. Guermazi A, De kerviler E, Cazals-hatem D et-al. Imaging findings in patients with myelofibrosis. Eur Radiol. 1999;9 (7): 1366-75. Eur Radiol (link) - Pubmed citation
- 3. Shaver RW, Clore FC. Extramedullary hematopoiesis in myeloid metaplasia. AJR Am J Roentgenol. 1981;137 (4): 874-6. AJR Am J Roentgenol (citation) - Pubmed citation
- 4. Choi H, David CL, Katz RL et-al. Case 69: extramedullary hematopoiesis. Radiology. 2004;231 (1): 52-6. doi:10.1148/radiol.2311020673 - Pubmed citation
- 5. Kaplan KR, Mitchell DG, Steiner RM et-al. Polycythemia vera and myelofibrosis: correlation of MR imaging, clinical, and laboratory findings. Radiology. 1992;183 (2): 329-34. Radiology (abstract) - Pubmed citation
- 6. Cloran F, Banks KP. AJR teaching file: Diffuse osteosclerosis with hepatosplenomegaly. AJR Am J Roentgenol. 2007;188 (3): S18-20. doi:10.2214/AJR.05.2141 - Pubmed citation
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