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Essential thrombocythaemia: challenges and evidence‐based management
Author(s) -
Harrison Claire N.
Publication year - 2005
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2005.05543.x
Subject(s) - myelofibrosis , medicine , hydroxycarbamide , myeloproliferative disorders , intensive care medicine , disease , etiology , anagrelide , ruxolitinib , polycythaemia , janus kinase 2 , dermatology , essential thrombocythemia , polycythemia vera , bone marrow , receptor
Summary 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.