Primary myelofibrosis

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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:

extramedullary hematopoiesis (EMH)
progressive splenomegaly
anaemia
variable change in the number of granulocytes and platelets including thrombocytopenia

Epidemiology

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

Pathology

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:

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~~haematopoiesis 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
  - ribs
  - proximal humerus and femur
- 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 the sinusoidal haematopoetic proliferation

Cardiovascular

may show evidence of congestive cardiac failure due to anaemia ³

Treatment and prognosis

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

gout: from hyperuricaemia due to increased haematopoietic turnover
splenic rupture (rare) ⁹
bleeding from thrombocytopenia (see case 8)

Differential diagnosis

General differential considerations include:

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

-</ul><h4>Radiographic features</h4><p>Most radiological features are a result of extramedullary haematopoeisis and seen in many systems.</p><h5>General</h5><ul><li><a href="/articles/lymph-node-enlargement">lymphadenopathy</a></li></ul><h5>Musculoskeletal</h5><ul><li>
+</ul><h4>Radiographic features</h4><p>Most radiological features are a result of extramedullary haematopoiesis and seen in many systems.</p><h5>General</h5><ul><li><a href="/articles/lymph-node-enlargement">lymphadenopathy</a></li></ul><h5>Musculoskeletal</h5><ul><li>
-</ul><h5>Cardiovascular</h5><ul><li>may show evidence of <a title="Congestive cardiac failure (CCF)" href="/articles/congestive-cardiac-failure">congestive </a><a title="Cardiac failure" href="/articles/heart-failure-basic">cardiac failure</a> due to anaemia <sup>3</sup>
+</ul><h5>Cardiovascular</h5><ul><li>may show evidence of <a href="/articles/congestive-cardiac-failure">congestive </a><a href="/articles/heart-failure-basic">cardiac failure</a> due to anaemia <sup>3</sup>

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