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

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)491-497
Number of pages7
JournalAmerican Journal of Hematology
Volume83
Issue number6
DOIs
StatePublished - Jun 2008

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Essential Thrombocythemia
Polycythemia Vera
Primary Myelofibrosis
Mutation
Therapeutics
Pharmaceutical Preparations
Neoplasms

ASJC Scopus subject areas

  • Hematology

Cite this

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abstract = "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.",
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