Citations 1
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, Francesco , Thiele, Jürgen, Girodon, François , Rumi, Elisa , Carobbio, Alessandra , Gisslinger, Heinz , Kvasnicka, Hans Michael , Ruggeri, Marco , Randi, Maria Luigia , Gangat, Naseema , Gianatti, Andrea , Gisslinger, Bettina, Müllauer, Leonhard , Rodeghiero, Francesco, d'Amore, Emanuele S. G. , Bertozzi, Irene , Hanson, Curtis A. , Boveri, Emanuela , Marino, Filippo, Maffioli, Margherita , Antonioli, Elisabetta , Carrai, Valentina , Buxhofer‐Ausch, Veronika , Pascutto, Cristiana, Cazzola, Mario , Barbui, Tiziano , Tefferi, Ayalew , Vannucchi, Alessandro M. , dʼAmore, Emanuele S.G. , Caramazza, Domenica , Passamonti, Francesco , Cazzola, Mario
Blood | June 27, 2012 | Volume: 120 | Issue: 6 | Pages: 1197-1201
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 × 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 × 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.