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Myelodysplastic syndrome

Incorporation of molecular data into the Revised International Prognostic Scoring System in treated patients with myelodysplastic syndromes

Abstract

The Revised International Prognostic Scoring System (IPSS-R) was developed for untreated myelodysplastic syndrome (MDS) patients based on clinical data. We created and validated a new model that incorporates mutational data to improve the predictive capacity of the IPSS-R in treated MDS patients. Clinical and mutational data from treated MDS patients diagnosed between January 2000 and January 2012 were used to develop the new prognostic system. A total of 508 patients were divided into training (n=333) and validation (n=175) cohorts. Independent significant prognostic factors for survival included age, IPSS-R, EZH2, SF3B1 and TP53. Weighted coefficients for each factor were used to build the new linear predictive model, which produced four prognostic groups: low, intermediate-1, intermediate-2 and high with a median overall survival of 37.4, 23.2, 19.9 and 12.2 months, respectively, P<0.001. Significant improvement in the C-index of the new model (0.73) was observed compared with the IPSS-R (0.69). The new model predicted outcome both in a separate validation cohort and in another cohort of patients with paired samples at different time points during their disease course. The addition of mutational data to the IPSS-R makes it dynamic and enhances its predictive ability in treated MDS patients regardless of their initial or subsequent therapies.

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Correspondence to M A Sekeres.

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The authors declare no conflict of interest.

Additional information

The study was presented as an oral presentation at the 57th annual meeting of the American Society of Hematology, Orlando, 2015.

Author contributions

NA, JPM and MAS designed the study, analyzed and interpreted the data, wrote and approved the manuscript. MN, TR, DJS, BP and BP collected the data, analyzed the samples, performed statistical analysis, reviewed and approved the manuscript. MK, AA and HEC contributed patients, analyzed the data, reviewed and approved the manuscript.

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Nazha, A., Narkhede, M., Radivoyevitch, T. et al. Incorporation of molecular data into the Revised International Prognostic Scoring System in treated patients with myelodysplastic syndromes. Leukemia 30, 2214–2220 (2016). https://doi.org/10.1038/leu.2016.138

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