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Leukemia. 2017 Dec;31(12):2726-2731. doi: 10.1038/leu.2017.169. Epub 2017 May 31.

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

Author information

1
Hematology, Department of Medicine and Surgery, University of Insubria, Ospedale di Circolo, ASST Sette Laghi, Varese, Italy.
2
Institute of Neurosciences, National Research Council of Italy, Padova, Italy.
3
CRIMM-Centro Ricerca e Innovazione delle Malattie Mieloproliferative, Department of Experimental and Clinical Medicine, Azienda ospedaliera-Universitaria Careggi, University of Florence, Florence, Italy.
4
Department of Hematology Oncology, Fondazione IRCCS Policlinico San Matteo, Università di Pavia, Pavia, Italy.
5
Department of Oncology-Hematology, University of Milan and BMT Unit, ASST Papa Giovanni XXIII, Bergamo, Italy.
6
Ospedale Niguarda Cà Granda, Milano, Italy.
7
Moffit Cancer Center, Tampa, FL, USA.
8
Stanford University, Palo Alto, CA, USA.
9
Hôpital Saint-Louis et Université Paris Diderot, Paris, France.
10
Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain.
11
Department of Hematology, University Hospitals Leuven and Laboratory of Experimental Transplantation, Department of Microbiology and Immunology, KU Leuven, Leuven, Belgium.
12
Policlinico S. Orsola-Malpighi, Bologna, Italy.
13
Università Cattolica del Sacro Cuore, Roma, Italy.
14
Ospedale S. Bortolo, Vicenza, Italy.
15
Weill Cornell Medical College, NY, USA.
16
SC Hematology, A.O. Città della Salute e della Scienza, Turin, Italy.
17
Università di Bari, Bari, Italy.
18
Cytogenetics and Medical Genetics Laboratory, Ospedale di Circolo, ASST Sette Laghi, Varese, Italy.
19
FROM Research Foundation, ASST Papa Giovanni XXIII, Bergamo, Italy.

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.

PMID:
28561069
DOI:
10.1038/leu.2017.169
[Indexed for MEDLINE]

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