Recent advances in pathogenesis and treatment of classical Philadelphia-negative myeloproliferative neoplasms
Journal Title: Postępy Nauk Medycznych - Year 2011, Vol 24, Issue 6
Abstract
In the last few years we have witnessed a great progress in our understanding of the pathogenesis of myeloproliferative neoplasms (MPNs). The classical Philadelphia-negative MPNs include polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). They were grouped together based on similar clinical phenotype by Dameshek in 1951. Since then the identification of JAK2V617F and MPL mutations has changed our view on disease pathogenesis and led to the revision of World Health Organization (WHO) diagnostic criteria for classical Philadelphia-negative MPNs. A new classification issued in 2008 incorporated these molecular abnormalities and therefore has modified the diagnostic approach. Moreover, these mutations have modified the strategies for monitoring and response assessment and they could become the potential targets for small-molecule agents. In this review we present current overview on molecular basis of MPNs including novel mutations and discuss the diagnostic criteria and therapeutic modalities.
Authors and Affiliations
Grzegorz Helbig, Sławomira Kyrcz-Krzemień
Keywords
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