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Br J Haematol. 2005 Jul;130(2):153-65.

Essential thrombocythaemia: challenges and evidence-based management.

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Department of Haematology, St Thomas Hospital, Lambeth Palace Road, London, UK.

Erratum in

  • Br J Haematol. 2005 Aug;130(3):465.


Essential thrombocythaemia was first described over 70 years ago. This condition is dominated by thrombotic and haemorrhagic complications and, in the long-term, by risk of transformation to myelofibrosis and/or acute leukaemia. However, it is heterogeneous both clinically and biologically. Here, a review of current concepts in disease aetiology and management is offered with reference to recent focused reviews where appropriate. In addition, five specific areas are discussed in detail: the role of the trephine biopsy, the disease entity prefibrotic myelofibrosis; the recently described Janus kinase 2 (JAK2) mutations; the leukaemogenicity of hydroxyurea (hydroxycarbamide); and lastly, the implications of the results of the Medical Research Council Primary Thrombocythaemia 1 study are explored.

[Indexed for MEDLINE]

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