title: The Wilms' tumor (WT1) gene expression correlates with the International Prognostic Scoring System (IPSS) score in patients with myelofibrosis and it is a marker of response to therapy.
abstract: The Wilms tumor gene WT1 is a useful marker of clonal hematopoiesis and it has been shown to be a good marker of residual disease and it reflects the response to therapy. Although myelofibrosis is characterized by mutations of JAK2 and calreticulin (CALR), these mutations are not useful to monitor response to therapy. In this study we demonstrated that in patients affected by myelofibrosis WT1 correlates with the International Prognostic Scoring System (IPSS) score at diagnosis. Furthermore WT1 is a good marker of response to JAK2 inhibitors especially for patients without blasts and for patients who develop anemia or thrombocytopenia not for progression but as therapy related toxicity. Finally, WT1 transcript reduction can mirror a benefit of therapy on the disease burden. This study demonstrated that WT1 is a good marker for monitoring the response to therapy in patients affected by myelofibrosis.
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Gallo
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Nicoli
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Calabrese
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Gaidano
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Jessica
authors.initials: J
authors.lastname: Petiti
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Rosso
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Signorino
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Sonia
authors.initials: S
authors.lastname: Carturan
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Giada
authors.initials: G
authors.lastname: Bot-Sartor
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Gisella
authors.initials: G
authors.lastname: Volpe
authors.affiliation: Department of Pediatric Hemato-Oncology and Stem Cell and Cellular Therapy Laboratory, Institute G. Gaslini, Largo G Gaslini, Genova.
authors.forename: Francesco
authors.initials: F
authors.lastname: Frassoni
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Saglio
authors.affiliation: Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Cilloni
mesh_terms: D015415:Biomarkers; D015870:Gene Expression; D006801:Humans; D053614:Janus Kinase 2 / Q000037:antagonists & inhibitors / Q000235:genetics; D009154:Mutation; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy; D011379:Prognosis; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D016896:Treatment Outcome; D025721:WT1 Proteins / Q000235:genetics*
chemical_list: D015415:Biomarkers; D047428:Protein Kinase Inhibitors; D025721:WT1 Proteins; C507924:JAK2 protein, human; D053614:Janus Kinase 2
keywords: JAK2; WT1; molecular marker; myelofibrosis