期刊
LEUKEMIA
卷 31, 期 12, 页码 2726-2731出版社
NATURE PUBLISHING GROUP
DOI: 10.1038/leu.2017.169
关键词
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资金
- Associazione Italiana per la Ricerca sul Cancro (AIRC
- Milano, Italy)
- Special Program Molecular Clinical Oncology 5 x 1000 [1005]
- AIRC [IG2014-15967]
- Ministero della Salute [GR-2011-02352109]
- Fondazione Matarelli (Milano, Italy)
- Fondazione Rusconi (Varese, Italy)
- AIL Varese ONLUS
- Fondazione Regionale Ricerca Biomedica (FRRB), Regione Lombardia
- Cancer Research and Treatment Fund, Inc., New York, NY
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 x 10(9)/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.
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