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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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