title: 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.
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 × 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 × 10(9)/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.
authors: Division of Hematology, Department of Internal Medicine, University Hospital Ospedale di Circolo e Fondazione Macchi, Varese, Italy.
authors: Francesco
authors: F
authors: Passamonti
authors: Jürgen
authors: J
authors: Thiele
authors: Francois
authors: F
authors: Girodon
authors: Elisa
authors: E
authors: Rumi
authors: Alessandra
authors: A
authors: Carobbio
authors: Heinz
authors: H
authors: Gisslinger
authors: Hans Michael
authors: HM
authors: Kvasnicka
authors: Marco
authors: M
authors: Ruggeri
authors: Maria Luigia
authors: ML
authors: Randi
authors: Naseema
authors: N
authors: Gangat
authors: Alessandro Maria
authors: AM
authors: Vannucchi
authors: Andrea
authors: A
authors: Gianatti
authors: Bettina
authors: B
authors: Gisslinger
authors: Leonhard
authors: L
authors: Müllauer
authors: Francesco
authors: F
authors: Rodeghiero
authors: Emanuele S G
authors: ES
authors: d'Amore
authors: Irene
authors: I
authors: Bertozzi
authors: Curtis A
authors: CA
authors: Hanson
authors: Emanuela
authors: E
authors: Boveri
authors: Filippo
authors: F
authors: Marino
authors: Margherita
authors: M
authors: Maffioli
authors: Domenica
authors: D
authors: Caramazza
authors: Elisabetta
authors: E
authors: Antonioli
authors: Valentina
authors: V
authors: Carrai
authors: Veronika
authors: V
authors: Buxhofer-Ausch
authors: Cristiana
authors: C
authors: Pascutto
authors: Mario
authors: M
authors: Cazzola
authors: Tiziano
authors: T
authors: Barbui
authors: Ayalew
authors: A
authors: Tefferi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D007391:International Cooperation; D008297:Male; D008875:Middle Aged; D015233:Models, Statistical; D015337:Multicenter Studies as Topic; D055728:Primary Myelofibrosis / Q000209:etiology / Q000628:therapy*; D011379:Prognosis; D016019:Survival Analysis; D013920:Thrombocythemia, Essential / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D014944:World Health Organization; D055815:Young Adult