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Essential thrombocythemia, polycythemia vera, and myelofibrosis: Current management and the prospect of targeted therapy
Author(s) -
Tefferi Ayalew
Publication year - 2008
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.21183
Subject(s) - essential thrombocythemia , myelofibrosis , polycythemia vera , medicine , sine qua non , myeloproliferative disorders , immunology , bone marrow , political science , law
The 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. © 2008 Wiley‐Liss, Inc.