Open AccessThe Wilms’ tumor ( WT 1 ) gene expression correlates with the International Prognostic Scoring System ( IPSS ) score in patients with myelofibrosis and it is a marker of response to therapy
Author(s) -
Gallo Daniela,
Nicoli Paolo,
Calabrese Chiara,
Gaidano Valentina,
Petiti Jessica,
Rosso Valentina,
Signorino Elisabetta,
Carturan Sonia,
BotSartor Giada,
Volpe Gisella,
Frassoni Francesco,
Saglio Giuseppe,
Cilloni Daniela
Publication year - 2016
Publication title -
cancer medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.403
H-Index - 53
ISSN - 2045-7634
DOI - 10.1002/cam4.735
Subject(s) - myelofibrosis , medicine , international prognostic scoring system , wilms' tumor , calreticulin , oncology , gastroenterology , cancer research , bone marrow , myelodysplastic syndromes , biology , biochemistry , endoplasmic reticulum
The Wilms tumor gene WT 1 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 JAK 2 and calreticulin ( CALR ), these mutations are not useful to monitor response to therapy. In this study we demonstrated that in patients affected by myelofibrosis WT 1 correlates with the International Prognostic Scoring System ( IPSS ) score at diagnosis. Furthermore WT 1 is a good marker of response to JAK 2 inhibitors especially for patients without blasts and for patients who develop anemia or thrombocytopenia not for progression but as therapy related toxicity. Finally, WT 1 transcript reduction can mirror a benefit of therapy on the disease burden. This study demonstrated that WT 1 is a good marker for monitoring the response to therapy in patients affected by myelofibrosis.