Abstract
Prognostic models are widely used in clinical practice for transplant decision-making in myelofibrosis (MF). We have compared the performance of the International Prognostic Scoring System (IPSS), dynamic IPSS (DIPSS), and DIPSS-plus in a series of 544 patients with primary or secondary MF aged ≤ 70 years at the time of diagnosis. The median projected survival of the overall series was 9.46 years (95% confidence interval 7.44–10.59). Median survival for the highest risk groups was less than 4 years in the three prognostic models. By contrast, the projected survival for patients in the intermediate-2 categories by the IPSS, DIPSS, and DIPSS-plus was 6.6, 5.6, and 6.5 years, respectively. The number of patients in the intermediate-2 and high-risk categories was smaller in the DIPSS than in the IPSS or the DIPSS-plus. The IPSS and DIPSS-plus were the best models to discriminate between the intermediate-1 and intermediate-2 risk categories, which is a critical cut-off point for patient selection to transplant. Among patients assigned at diagnosis to the intermediate-2 or high-risk groups by the IPSS, DIPSS, and DIPSS-plus, only 17, 21, and 20%, respectively, were subsequently transplanted. In conclusion, in our contemporary series of younger MF patients only the highest risk categories of the current prognostication systems have a median survival below the 5-year threshold recommended for considering transplantation. Patient selection for transplantation can significantly differ depending on which prognostication model is used for disease risk stratification.
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Acknowledgments
This work was supported by the grant RD12/0036/0010 from the Instituto de Salud Carlos III, Spanish Ministry of Health. We are indebted to all members of GEMFIN participating in the Spanish Registry of Myelofibrosis.
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JCHB designed the research, performed the statistical analysis, interpreted the results, and wrote the manuscript. APe performed the statistical analysis, interpreted the results, and wrote the manuscript. JGC, AAL, FFM, JMR, JML, PV, MPE, NE, VGG, MLF, APa, AK, BC, MAD, MJR, MTGC, MIMV, EM, and MG collected the data, revised the manuscript, and approved the final version of the manuscript. FC interpreted the results and wrote the manuscript.
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Hernández-Boluda, JC., Pereira, A., Correa, JG. et al. Prognostic risk models for transplant decision-making in myelofibrosis. Ann Hematol 97, 813–820 (2018). https://doi.org/10.1007/s00277-018-3240-x
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DOI: https://doi.org/10.1007/s00277-018-3240-x