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 10(9)/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 10(9)/L, and history of thrombosis allows prognostic assessment of
A prognostic model to predict survival in 867 World Health Organization-defined essential thrombocythemia at diagnosis: a study by the International Working Group on Myelofibrosis Research and Treatment / Passamonti, F; Thiele, J; Girodon, F; Rumi, E; Carobbio, A; Gisslinger, H; Kvasnicka, Hm; Ruggeri, M; Randi, Ml; Gangat, N; Vannucchi, Am; Gianatti, A; Gisslinger, B; Mullauer, L; Rodeghiero, F; D'Amore, Es; Bertozzi, I; Hanson, Ca; Boveri, E; Marino, F; Maffioli, M; Caramazza, D; Antonioli, E; Carrai, V; Buxhofer-Ausch, V; Pascutto, C; Cazzola, M; Barbui, T; Tefferi, A. - In: BLOOD. - ISSN 1528-0020. - 120:6(2012), pp. 1197-1201. [10.1182/blood-2012-01-403279]
A prognostic model to predict survival in 867 World Health Organization-defined essential thrombocythemia at diagnosis: a study by the International Working Group on Myelofibrosis Research and Treatment
Carobbio A;
2012
Abstract
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 10(9)/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 10(9)/L, and history of thrombosis allows prognostic assessment of
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