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Cited by 277

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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

Open access
Passamonti, Francesco, Thiele, Jürgen, 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, Müllauer, 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, Buxhofer-Ausch, Veronika, Pascutto, Cristiana, Cazzola, Mario, Barbui, Tiziano, Tefferi, Ayalew·2012·Blood

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.

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Metadata

Authors
Passamonti, F., Thiele, J., Girodon, F., Rumi, E., Carobbio, A., Gisslinger, H., Kvasnicka, H. M., Ruggeri, M., Randi, M. L., Gangat, N., Vannucchi, A. M., Gianatti, A., Gisslinger, B., Müllauer, L., Rodeghiero, F., d'Amore, E. S. G., Bertozzi, I., Hanson, C. A., Boveri, E., Marino, F., Maffioli, M., Caramazza, D., Antonioli, E., Carrai, V., Buxhofer-Ausch, V., Pascutto, C., Cazzola, M., Barbui, T., & Tefferi, A.
Venue
Blood
Published
2012-08-09
Volume
120
Issue
6
Pages
1197-1201
ISSN
  • 0006-4971 (ISSN-L)
  • 1528-0020
OpenAlex
W1985994644
Cited by
277
References
20
FWCI
10.27
Type
journal article
Language
en
Open access
bronze

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CoChat. (2026). Citation check: 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. https://app.cochat.ai/papers/10.1182/blood-2012-01-403279
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Passamonti, F., Thiele, J., Girodon, F., Rumi, E., Carobbio, A., Gisslinger, H., Kvasnicka, H. M., Ruggeri, M., Randi, M. L., Gangat, N., Vannucchi, A. M., Gianatti, A., Gisslinger, B., Müllauer, L., Rodeghiero, F., d'Amore, E. S. G., Bertozzi, I., Hanson, C. A., Boveri, E., . . . Tefferi, A. (2012). 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. Blood. https://doi.org/10.1182/blood-2012-01-403279

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.