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

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...

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Veröffentlicht in:	Leukemia 2017-12, Vol.31 (12), p.2726-2731
Hauptverfasser:	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
Format:	Artikel
Sprache:	eng
Schlagworte:	692/4028/67/70 692/499 692/699/1541/1990/2331 692/700/1750 Adult Aged Aged, 80 and over Biomarkers Blood Blood cancer Blood platelets Cancer Research Care and treatment Clinical decision making Critical Care Medicine Decision making Development and progression Female Follow-Up Studies Genetic aspects Genotype & phenotype Genotypes Hematology Hemoglobin Humans Intensive Internal Medicine Janus kinase 2 Laboratories Leukemia Male Mathematical models Medicine Medicine & Public Health Methods Middle Aged Mutation Myelofibrosis Neoplasms Oncology original-article Patients Polycythemia Polycythemia vera Polycythemia Vera - diagnosis Polycythemia Vera - genetics Polycythemia Vera - mortality Primary Myelofibrosis - diagnosis Primary Myelofibrosis - genetics Primary Myelofibrosis - mortality Prognosis Regression analysis Risk Risk Factors Statistical models Stem cell transplantation Survival Survival Analysis Thrombocythemia Thrombocythemia, Essential - diagnosis Thrombocythemia, Essential - genetics Thrombocythemia, Essential - mortality Thrombocytopenia Thrombocytosis Thrombosis Tumors
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Zusammenfassung:	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
ISSN:	0887-6924 1476-5551
DOI:	10.1038/leu.2017.169