期刊
BLOOD
卷 120, 期 6, 页码 1197-1201出版社
AMER SOC HEMATOLOGY
DOI: 10.1182/blood-2012-01-403279
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资金
- Associazione Italiana contro Leucemie (AIL
- Linfomi, Mieloma) Varese Onlus
- Associazione Italiana per la Ricerca sul Cancro (Special Program Molecular Clinical Oncology 5x1000) [1005]
- Fondazione Cariplo
- Regione Lombardia
- Italian Ministry of Health
- Associazione Italiana per la Ricerca sul Cancro (AIRC
- Milano, Italy) Special Program Molecular Clinical Oncology 5x1000 [1005]
Diagnosis of essential thrombocythemia (ET) has been updated in the last World Health Organization (WHO) classification. We developed a prognostic model to predict survival at diagnosis, named IPSET (International Prognostic Score for ET), studying patients with WHO-defined ET. Age 60 years or older, leukocyte count >= 11 x 109/L, and prior thrombosis significantly affected survival, by multivariable Cox regression. On the basis of the hazard ratio, we assigned 2 points to age and 1 each to leukocyte count and thrombosis. So, the IPSET model allocated 867 patients into 3 risk categories with significantly different survival: low (sum of points = 0; median survival not reached), intermediate (sum = 1-2; median survival 24.5 years), and high (sum = 3-4, median survival 13.8 years). The IPSET model was further validated in 2 independent cohorts including 132 WHO-defined ET and 234 Polycythemia Vera Study Group-defined ET patients. The IPSET model was able to predict the occurrence of thrombosis, and not to predict post-ET myelofibrosis. In conclusion, IPSET, based on age >= 60 years, leukocyte count >= 11 x 109/L, and history of thrombosis allows prognostic assessment of WHO-defined ET and the validation process makes IPSET applicable in all patients phenotypically appearing as ET. (Blood. 2012;120(6):1197-1201)
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