Essential thrombocythaemia: challenges and evidence-based management.
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.
Harrison
Claire N
CN
Department of Haematology, St Thomas Hospital, Lambeth Palace Road, London, UK. claire.harrison@gstt.nhs.uk
D019317:Evidence-Based Medicine; D006801:Humans; D016032:Randomized Controlled Trials as Topic; D013920:Thrombocythemia, Essential / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000235:genetics