Practice-relevant revision of IPSET-thrombosis based on 1019 patients with WHO-defined essential thrombocythemia

Authors: Tiziano Barbui, Alessandro M. Vannucchi, Veronika Buxhofer‐Ausch, Valerio De Stefano, S Betti, Alessandro Rambaldi, Elisa Rumi, Marco Ruggeri, Francesco Rodeghiero, Maria Luigia Randi, Irene Bertozzi, Heinz Gisslinger, Guido Finazzi, Alessandra Carobbio, J. Thiele, Francesco Passamonti, Chiara Falcone, Ayalew Tefferi - Blood Cancer Journal 2015 cited by 268

Current risk stratification for thrombosis in essential thrombocythemia (ET) is two-tiered and considers low- and high-risk categories based on the respective absence or presence of either age >60 years or history of thrombosis. 1 In the recent International Prognostic Score for Thrombosis in ET (IPSET-thrombosis), age and history of thrombosis were confirmed as independent risk factors for future thrombosis and the study also identified independent prothrombotic role for cardiovascular (CV) risk factors and JAK2 V617F mutation. 2 This model outperformed the two-tiered conventional risk stratification in predicting future vascular events and was not further affected by the recently discovered CALR mutation. 3 In the current study, we re-analyzed the original IPSET-thrombosis data in 1019 patients with WHO-defined ET in whom JAK2 mutational status was available, to quantify the individual contributions of JAK2 mutations and CV risk factors in conventionally assigned low- and high-risk ET.