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Myelofibrosis

Dr Yuranga Weerakkody et al.

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.

Epidemiology

It usually affects the middle aged to elderly (mean age 60 ⁶). The estimated prevalence is at ~ 1 : 100,000.

Sub types

It can be broadly classified into

primary myelofibrosis - e.g. agnogenic myeloid metaplasia
secondary myelofibrosis - marrow replacement from malignancy / toxins

Radiographic features

Most radiological features are a result of extramedullary haematopoeisis (EMH) and seen in many systems

General

lymphadenopathy

Musculoskeletal

osteosclerosis
- tends to be diffuse and there is a lack of architectural distortion
- frequent sites include
  - axial skeleton (insive of pelvis and skull)
  - ribs
  - proximal humerus and femur.
- a bone scan may give "superscan" appearance

Abdominal

hepatomegaly
splenomegaly - can be massive
evidence of portal hypertension ³
- from increased splenic blood flow
- from portal flow obstruction from sinusoidal haematopoetic proliferation

Cardiovascular

may show evidence of congestive cardiac failure due to anaemia ³.

Complications

gout : from hyperuricaemia due to increased haematopoeitic turnover

Differential diagnosis

General differential considerations include

for musculoskeletal manifestations - consider differential diagnosis of diffuse bony sclerosis
for splenic manifestations - consider differential diagnosis for splenomegaly

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