TY - JOUR
T1 - A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis
AU - Passamonti, F.
AU - Giorgino, T.
AU - Mora, B.
AU - Guglielmelli, P.
AU - Rumi, E.
AU - Maffioli, M.
AU - Rambaldi, A.
AU - Caramella, M.
AU - Komrokji, R.
AU - Gotlib, J.
AU - Kiladjian, J. J.
AU - Cervantes, F.
AU - Devos, T.
AU - Palandri, F.
AU - De Stefano, Valerio
AU - Ruggeri, M.
AU - Silver, R. T.
AU - Benevolo, G.
AU - Albano, F.
AU - Caramazza, D.
AU - Merli, M.
AU - Pietra, D.
AU - Casalone, R.
AU - Rotunno, G.
AU - Barbui, T.
AU - Cazzola, M.
AU - Vannucchi, A. M.
PY - 2017
Y1 - 2017
N2 - 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 o 11 g/dl, to circulating blasts 3%, and to CALR-unmutated genotype, 1 point to platelet count o 150 Ã 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 o 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: 3.2â7.9; 126 patients), and high risk (2 years, 95% CI: 1.7â3.9; 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.
AB - 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 o 11 g/dl, to circulating blasts 3%, and to CALR-unmutated genotype, 1 point to platelet count o 150 Ã 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 o 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: 3.2â7.9; 126 patients), and high risk (2 years, 95% CI: 1.7â3.9; 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.
KW - Cancer Research, Secondary Myelofibrosis, Prognostic Model
KW - Cancer Research, Secondary Myelofibrosis, Prognostic Model
UR - http://hdl.handle.net/10807/114799
UR - http://www.nature.com/leu/index.html
U2 - 10.1038/leu.2017.169
DO - 10.1038/leu.2017.169
M3 - Article
SN - 0887-6924
VL - 31
SP - 2726
EP - 2731
JO - Leukemia
JF - Leukemia
ER -
