title: Oncogenic signals as treatment targets in classic myeloproliferative neoplasms.
abstract: Therapeutically validated oncoproteins in myeloproliferative neoplasms (MPNs) include BCR-ABL in chronic myelogenous leukemia (CML) and a spectrum of PDGFRA/B mutant proteins that are products of intra- (eg, FIP1L1-PDGFRA) or interchromosomal (eg, ETV6-PDGFRB) gene fusions. Other MPN-relevant putative oncogenes that are awaiting therapeutic validation, include JAK2 and MPL mutations in polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF); KITD816V and other KIT mutations in systemic mastocytosis, and FGFR1 rearrangements associated with the 8p11 leukemia/lymphoma syndrome. The current review focuses on mutant molecules of interest in classic MPNs (ie, CML, PV, ET, and PMF) in the context of their value as drug targets.
authors.affiliation: Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
authors.forename: Ross L
authors.initials: RL
authors.lastname: Levine
authors.forename: Hagop
authors.initials: H
authors.lastname: Kantarjian
mesh_terms: D016503:Drug Delivery Systems; D006801:Humans; D009196:Myeloproliferative Disorders / Q000188:drug therapy* / Q000235:genetics*; D015513:Oncogene Proteins; D015514:Oncogene Proteins, Fusion; D015398:Signal Transduction / Q000235:genetics
chemical_list: D015513:Oncogene Proteins; D015514:Oncogene Proteins, Fusion