Essential thrombocythemia, polycythemia vera, and myelofibrosis: current management and the prospect of targeted therapy.

摘要: 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 treat these disorders. For instance, the presence mutation is now considered conditio sine qua non for diagnosis PV World Health Organization classification system recently revised its diagnostic criteria PV, ET, PMF to include as clonal markers. From standpoint treatment, JAK-STAT identified legitimate target pathway drug development myeloproliferative neoplasms. Herein, I will first outline my views regarding current management then discuss emerging data preclinical clinical activity anti-JAK2 small molecule drugs. Am. J. Hematol., 2008. (c) 2008 Wiley-Liss, Inc.