Myelofibrosis
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  • Marrow fibrosis and splenomegaly, de novo or following transformation of PV or ET
  • Usually >50 years old
  • Abnormal megkaryocytes produced in increased numbers.
  • PDGF and TGFβ are released by megakaryocytes, stimulating fibrosis.
  • Haematopoietic stem cells move to the spleen and liver
  • Presentation: fatigue, weight loss, splenomegaly, splenic pain, portal hypertension, bleeding varices, ascites and hepatomegaly
  • Anaemia and tear-drop poikilocytes (abnormally shaped RBCs)
  • Initially thrombocytosis and neutrophilia, in advanced disease these fall
  • Bone marrow: aspiration gives dry tap, trephine shows dense reticulin fibres, fibrosis and osteosclerosis
  • JAK2 mutation seen in 40%
  • Average survival 5-7 years, leukaemic transformation in 15%
  • Asymptomatic cases may require no treatment
  • Anaemia treated with corticosteroid, androgen or EPO – transfusion usually required
  • Hydroxycarbamide reduces symptoms and splenomegaly
  • Thalidomide: counter abnormal marrow angiogenesis
  • Splenic irradiation and splenectomy may be considered
  • Allogenic SCT potentially curative, limited to rare, young patients

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Dr Tom Leach

Dr Tom Leach MBChB DCH EMCert(ACEM) currently works as a GP Registrar and an Emergency Department CMO in Australia. He is also a Clinical Associate Lecturer at the Australian National University. After graduating from his medical degree at the University of Manchester in 2011, Tom completed his Foundation Training at Bolton Royal Hospital, before moving to Australia in 2013. He started almostadoctor whilst a third year medical student in 2009. Read full bio

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