Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms with variable risk of evolution into post-PV and post-ET myelofibrosis, from now on referred to as secondary myelofibrosis (SMF). No specific tools have been defined for risk stratification in SMF. To develop a prognostic model for predicting survival, we studied 685 JAK2, CALR, and MPL annotated patients with SMF. Median survival of the whole cohort was 9.3 years (95% CI: 8-not reached-NR-). Through penalized Cox regressions we identified negative predictors of survival and according to beta risk coefficients we assigned 2 points to hemoglobin level <11 g/dl, to circulating blasts ⩾3%, and to CALR-unmutated genotype, 1 point to platelet count <150 × 109/l and to constitutional symptoms, and 0.15 points to any year of age. Myelofibrosis Secondary to PV and ET-Prognostic Model (MYSEC-PM) allocated SMF patients into four risk categories with different survival (P<0.0001): low (median survival NR; 133 patients), intermediate-1 (9.3 years, 95% CI: 8.1-NR; 245 patients), intermediate-2 (4.4 years, 95% CI: 3.2-7.9; 126 patients), and high risk (2 years, 95% CI: 1.7-3.9; 75 patients). Finally, we found that the MYSEC-PM represents the most appropriate tool for SMF decision-making to be used in clinical and trial settings.

A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis / Passamonti, F.; Giorgino, T.; Mora, B.; Guglielmelli, P.; Rumi, E.; Maffioli, M.; Rambaldi, A.; Caramella, M.; Komrokji, R.; Gotlib, J.; Kiladjian, J. J.; Cervantes, F.; Devos, T.; Palandri, F.; De Stefano, V.; Ruggeri, M.; Silver, R. T.; Benevolo, G.; Albano, F.; Caramazza, D.; Merli, M.; Pietra, D.; Casalone, R.; Rotunno, G.; Barbui, T.; Cazzola, M.; Vannucchi, A. M.. - In: LEUKEMIA. - ISSN 0887-6924. - ELETTRONICO. - 31:(2017), pp. 2726-2731. [10.1038/leu.2017.169]

A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis

Guglielmelli, P.;Rotunno, G.;Vannucchi, A. M.
2017

Abstract

Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms with variable risk of evolution into post-PV and post-ET myelofibrosis, from now on referred to as secondary myelofibrosis (SMF). No specific tools have been defined for risk stratification in SMF. To develop a prognostic model for predicting survival, we studied 685 JAK2, CALR, and MPL annotated patients with SMF. Median survival of the whole cohort was 9.3 years (95% CI: 8-not reached-NR-). Through penalized Cox regressions we identified negative predictors of survival and according to beta risk coefficients we assigned 2 points to hemoglobin level <11 g/dl, to circulating blasts ⩾3%, and to CALR-unmutated genotype, 1 point to platelet count <150 × 109/l and to constitutional symptoms, and 0.15 points to any year of age. Myelofibrosis Secondary to PV and ET-Prognostic Model (MYSEC-PM) allocated SMF patients into four risk categories with different survival (P<0.0001): low (median survival NR; 133 patients), intermediate-1 (9.3 years, 95% CI: 8.1-NR; 245 patients), intermediate-2 (4.4 years, 95% CI: 3.2-7.9; 126 patients), and high risk (2 years, 95% CI: 1.7-3.9; 75 patients). Finally, we found that the MYSEC-PM represents the most appropriate tool for SMF decision-making to be used in clinical and trial settings.
2017
31
2726
2731
Goal 3: Good health and well-being for people
Passamonti, F.; Giorgino, T.; Mora, B.; Guglielmelli, P.; Rumi, E.; Maffioli, M.; Rambaldi, A.; Caramella, M.; Komrokji, R.; Gotlib, J.; Kiladjian, J. J.; Cervantes, F.; Devos, T.; Palandri, F.; De Stefano, V.; Ruggeri, M.; Silver, R. T.; Benevolo, G.; Albano, F.; Caramazza, D.; Merli, M.; Pietra, D.; Casalone, R.; Rotunno, G.; Barbui, T.; Cazzola, M.; Vannucchi, A. M.
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1106622
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