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

作者：Passamonti Francesco^*; Thiele Juergen; Girodon Francois; Rumi Elisa; Carobbio Alessandra; Gisslinger Heinz; Kvasnicka Hans Michael; Ruggeri Marco; Randi Maria Luigia; Gangat Naseema; Vannucchi Alessandro Maria; Gianatti Andrea; Gisslinger Bettina; Muellauer Leonhard; Rodeghiero Francesco; d' Amore Emanuele S G; Bertozzi Irene; Hanson Curtis A; Boveri Emanuela; Marino Filippo; Maffioli Margherita; Caramazza Domenica; Antonioli Elisabetta; Carrai Valentina

来源：Blood, 2012, 120(6): 1197-1201.

DOI：10.1182/blood-2012-01-403279

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 %26gt;= 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 %26gt;= 60 years, leukocyte count %26gt;= 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)

• 出版日期2012-8-9