Primary myelofibrosis
Updates to Article Attributes
Primary myelofibrosis is a myeloproliferative neoplasm in which there is the replacement of bone marrow with collagenous connective tissue and progressive fibrosis. It is characterised by:
- extramedullary hematopoiesis
- 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 years 6. The estimated prevalence is ~1:100,000.
Pathology
It is considered a chronic BCR-ABL1 (breakpoint cluster region-Abelson murine leukemia viral oncogene homologue 1)-negative myeloproliferative disorder 11.
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.
Radiographic features
Most radiological features are a result of extramedullary haematopoiesis and seen in many systems.
General
Musculoskeletal
Abdominal
- hepatomegaly
-
splenomegaly: can be massive
- some patients may also experience splenic infarcts 11
- evidence of portal hypertension 3
- from increased splenic blood flow
- from portal flow obstruction from the sinusoidal
haematopoetichaematopoietic 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 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
See also
-<p><strong>Primary myelofibrosis</strong> is a <a title="Myeloproliferative neoplasm" href="/articles/myeloproliferative-neoplasm-1">myeloproliferative neoplasm</a> in which there is the replacement of <a href="/articles/bone-marrow">bone marrow</a> with collagenous connective tissue and progressive fibrosis. It is characterised by:</p><ul>- +<p><strong>Primary myelofibrosis</strong> is a <a href="/articles/myeloproliferative-neoplasm-1">myeloproliferative neoplasm</a> in which there is the replacement of <a href="/articles/bone-marrow">bone marrow</a> with collagenous connective tissue and progressive fibrosis. It is characterised by:</p><ul>
-<li>bone scan may give "<a href="/articles/superscan">superscan</a>" appearance</li>- +<li>
- +<a title="Bone scan" href="/articles/bone-scan">bone scan</a> may give "<a href="/articles/superscan">superscan</a>" appearance</li>
-<li>from portal flow obstruction from the sinusoidal haematopoetic proliferation</li>- +<li>from portal flow obstruction from the sinusoidal haematopoietic proliferation</li>
-</li></ul><h4>Treatment and prognosis</h4><p>Prognosis is poor, with slow progression and death usually within 2-3 years. It can also transform into acute myeloid leukaemia in a small number of patients <sup>10</sup>.</p><h5>Complications</h5><ul>- +</li></ul><h4>Treatment and prognosis</h4><p>Prognosis is poor, with slow progression and death usually within 2-3 years. It can also transform into <a title="Acute myeloid leukaemia" href="/articles/acute-myeloid-leukaemia">acute myeloid leukaemia</a> in a small number of patients <sup>10</sup>.</p><h5>Complications</h5><ul>
-</ul><h4>See also</h4><ul><li><a title="WHO classification of tumours of haematopoietic and lymphoid tissues" href="/articles/who-classification-of-tumours-of-haematopoietic-and-lymphoid-tissues-1">WHO classification of tumours of haematopoietic and lymphoid tissues</a></li></ul>- +</ul><h4>See also</h4><ul><li><a href="/articles/who-classification-of-tumours-of-haematopoietic-and-lymphoid-tissues-1">WHO classification of tumours of haematopoietic and lymphoid tissues</a></li></ul>
Image 10 X-ray (Fronal pelvis radiograph) ( create )
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