TY - JOUR
T1 - A prognostic model to predict survival in 867 World Health Organization - Defined essential thrombocythemia at diagnosis
T2 - A study by the International Working Group on Myelofibrosis Research and Treatment
AU - Passamonti, Francesco
AU - Thiele, Jürgen
AU - Girodon, Francois
AU - Rumi, Elisa
AU - Carobbio, Alessandra
AU - Gisslinger, Heinz
AU - Kvasnicka, Hans Michael
AU - Ruggeri, Marco
AU - Randi, Maria Luigia
AU - Gangat, Naseema
AU - Vannucchi, Alessandro Maria
AU - Gianatti, Andrea
AU - Gisslinger, Bettina
AU - Müllauer, Leonhard
AU - Rodeghiero, Francesco
AU - D'Amore, Emanuele S.G.
AU - Bertozzi, Irene
AU - Hanson, Curtis A.
AU - Boveri, Emanuela
AU - Marino, Filippo
AU - Maffioli, Margherita
AU - Caramazza, Domenica
AU - Antonioli, Elisabetta
AU - Carrai, Valentina
AU - Buxhofer-Ausch, Veronika
AU - Pascutto, Cristiana
AU - Cazzola, Mario
AU - Barbui, Tiziano
AU - Tefferi, Ayalew
PY - 2012/8/9
Y1 - 2012/8/9
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84865187605&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84865187605&partnerID=8YFLogxK
U2 - 10.1182/blood-2012-01-403279
DO - 10.1182/blood-2012-01-403279
M3 - Article
C2 - 22740446
AN - SCOPUS:84865187605
SN - 0006-4971
VL - 120
SP - 1197
EP - 1201
JO - Blood
JF - Blood
IS - 6
ER -