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 Essential thrombocythemia, polycythemia vera, and myelofibrosis: current  management and the prospect of targeted therapy Tefferi AAm J Hematol  2008[Jun]; 83 (6): 491-7The recent discovery of JAK2 and/or MPL mutations in polycythemia vera (PV),  essential thrombocythemia (ET), and primary myelofibrosis (PMF) has had a major  impact on how we diagnose and treat these disorders. For instance, the presence  of a JAK2 mutation is now considered conditio sine qua non for the diagnosis of  PV and the World Health Organization classification system has recently revised  its diagnostic criteria for PV, ET, and PMF to include JAK2 and MPL mutations as  clonal markers. From the standpoint of treatment, JAK-STAT is now identified as a  legitimate target pathway for drug development in myeloproliferative neoplasms.  Herein, I will first outline my views regarding current management in ET, PV, and  PMF and then discuss emerging data on preclinical and clinical activity of  anti-JAK2 small molecule drugs. Am. J. Hematol., 2008. (c) 2008 Wiley-Liss, Inc.|Disease Management[MESH]|Drug Delivery Systems[MESH]|Humans[MESH]|Janus Kinase 2/genetics[MESH]|Myeloproliferative Disorders/*classification/diagnosis/*drug therapy[MESH]|Polycythemia Vera/classification/diagnosis/drug therapy[MESH]|Primary Myelofibrosis/classification/diagnosis/drug therapy[MESH]|Receptors, Thrombopoietin/genetics[MESH]|Thrombocythemia, Essential/classification/diagnosis/drug therapy[MESH]
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