期刊
BRITISH JOURNAL OF HAEMATOLOGY
卷 130, 期 2, 页码 153-165出版社
WILEY
DOI: 10.1111/j.1365-2141.2005.05543.x
关键词
essential thrombocythaemia; hydroxyurea; trephine biopsy; MRC-PT1
类别
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.
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