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Correlation between IPSET-t risk at diagnosis and subsequent hemorrhage in patients with essential thrombocythemia; a single institution experience

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Abstract

Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by an increased risk of thrombotic and hemorrhagic events, that represent the leading causes of mortality and morbidity. Currently, while thrombotic risk is assessed through the IPSET-t and r-IPSET scores, there is no specific prognostic tool used to predict hemorrhagic risk in ET. The aim of the study was to define incidence and risk factors connected to hemorrhagic events by retrospectively analyzing 308 ET patients diagnosed between 1996 and 2022 at the Division of Hematology of Udine and treated according to the current international guidelines. According to molecular status, 193 patients (62.7%) were JAK2 mutated, 66 (21.4%) had a CALR mutation, 14 (4.5%) had a MPL mutation, 21 patients (6.8%) were “triple negative,” and 14 patients (4.5%) were not evaluable. According to IPSET-t score, 49.7% patients were at high, 24.3% at intermediate, and 26.0% at low-risk, respectively. Twelve (3.9%) patients experienced bleeding at ET diagnosis, while 24 (7.8%) had at least one hemorrhagic event during follow-up at a median time of 103 months (range: 1–309). Forty hemorrhagic events were totally recorded and defined as minor in 22 cases, moderate in 11 cases, and severe in 7 cases. Cumulative incidence (CI) of hemorrhage at 10 and 20 years was 6.0% and 12.0%, respectively. A statistically significant correlation between hemorrhagic risk and IPSET-t score emerged: 10 years hemorrhage CI was 3.2% for low-risk, 2.9% for intermediate-risk, and 9.8% for high-risk patients, respectively (p=0.002). We found no correlation between hemorrhagic risk and gender or mutational status. Results of our study highlight the validity of IPSET-t score in predicting individual hemorrhagic risk among ET patients, suggesting a possible role of IPSET-t scoring system as a global evaluator for vascular events in ET patients.

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Authors and Affiliations

  1. Division of Hematology and Bone Marrow Transplant, Azienda Sanitaria Universitaria Friuli Centrale, P.le S. M. Misericordia 15, 33100, Udine, Italy

    Luca Tosoni, Matteo Liberi, Gianluca Morelli, Maria Elena Zannier, Davide Lazzarotto, Carla Filì, Erica Simeone, Giulia Battaglia, Chiara Callegari, Matteo Fanin, Daniela Damiani, Renato Fanin & Mario Tiribelli

  2. Department of Medicine, University of Udine, Udine, Italy

    Daniela Damiani, Renato Fanin & Mario Tiribelli

Authors
  1. Luca Tosoni
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  2. Matteo Liberi
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  3. Gianluca Morelli
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  4. Maria Elena Zannier
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  5. Davide Lazzarotto
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  6. Carla Filì
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  7. Erica Simeone
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  8. Giulia Battaglia
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  9. Chiara Callegari
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  10. Matteo Fanin
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  11. Daniela Damiani
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  12. Renato Fanin
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  13. Mario Tiribelli
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Contributions

LT and MT conceived the study, analyzed the data, and wrote the paper; GM, MEZ, DL, CF, ES, GB, CG, and MF collected data; DD and RF critically revised the paper.

Corresponding author

Correspondence to Mario Tiribelli.

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Obtained by the IRB of the Department of Medicine, University of Udine (PROT IRB 50/2023).

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The authors declare no competing interests.

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The original version of this article was revised: This article was originally published without a missing author name Matteo Liberi1.

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Tosoni, L., Liberi, M., Morelli, G. et al. Correlation between IPSET-t risk at diagnosis and subsequent hemorrhage in patients with essential thrombocythemia; a single institution experience. Ann Hematol 103, 443–448 (2024). https://doi.org/10.1007/s00277-023-05578-8

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  • DOI: https://doi.org/10.1007/s00277-023-05578-8

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