WT1 gene is overexpressed in myeloproliferative neoplasms, especially in myelofibrosis

Publication date: Available online 20 December 2018Source: Blood Cells, Molecules, and DiseasesAuthor(s): Laurane Cottin, Jérémie Riou, Françoise Boyer, Anne Bouvier, Alain Zannetti, Anaïse Blouet, Matgorzata Truchan-Graczyk, Rébecca Jouanneau-Courville, Annaëlle Beucher, Bénédicte Ribourtout, Corentin Orvain, Mathilde Hunault-Berger, Odile Blanchet, Valérie Ugo, Damien Luque PazAbstractClassical Philadelphia-negative myeloproliferative neoplasms include Polycythemia Vera (PV), Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF). They are characterized by the presence of driver mutations of JAK2, CALR or MPL genes. Overexpression of WT1 is used as a marker of minimal residual disease in acute myeloid leukemia, especially after allogeneic stem cell transplantation (SCT). We investigated WT1 expression at diagnosis in 152 MPN patients and showed that the WT1 transcript was overexpressed in PMFs and PVs compared to controls. In particular, WT1 transcript levels were higher in PMF than in ET and PV. WT1 transcript levels were significantly increased during myelofibrotic transformation of ET or PV. Using multivariate linear regression, high WT1 transcript levels in PMF were associated with age over 65, splenomegaly and thrombocytopenia. The ROC curve analysis showed that a level of WT1 transcript>10 WT1 copies/104ABL1 enabled the diagnosis of PMF with a specificity of 95.8% (PMF vs ET; ROC AUC = 0.91). In myelofibrosis, studying follow-ups of WT1 transcript...
Source: Blood Cells, Molecules, and Diseases - Category: Hematology Source Type: research