Comparison between thrombotic risk scores in essential thrombocythemia and survival implications

Abstract

The conventional thrombotic risk stratification in essential thrombocythemia (ET) distinguishes patients in two risk groups based on previous thrombosis and age (< or >60). The IPSET‐thrombosis takes into account four risk factors: age greater than 60 years and the presence of CV risk factors, thrombosis history and JAK2 V617F presence. The revised IPSET‐thrombosis uses three adverse variables to delineate four risk categories: age greater than 60, thrombosis history, and JAK2 V617F presence. We compared different risk models in the estimation of thrombotic risk in 191 patients with ET and the role of specific driver mutations affecting overall survival, according to thrombotic risk. We also evaluated the mutational status of patients showing history of thrombosis or cardiovascular events versus patients who did not. Finally, we verified whether the thrombotic risk had a significant impact on survival in our ET patients. The data analysis has been performed through the conventional statistics and overall survival estimated by using the Kaplan‐Meyer method. Interestingly, either using the traditional system for thrombotic risk or the IPSET‐t prognostic score or the current stratification for the thrombotic risk, high‐risk patients are always highly represented. This evidence is of note, being the high‐risk category indicated for cytoreduction, affecting quality of life, despite the good overall prognosis of patients with ET diagnosis in general. The analysis of overall survival in our patients, according to different models for thrombotic risk, highlighted the poor prognosis of high‐risk patients compared with those with a lower thrombotic risk, in particular when using traditional stratification and current stratification. In conclusion, the occurrence of thrombotic or cardiovascular events represents one of the most severe complications at diagnosis or during follow‐up of ET despite current recommendations, having a significant impact on morbidity and survival.

The Essential Thrombocythemia in 2020: What We Know and Where We Still Have to Dig Deep

“…This data confirm previous experiences showing, through retrospective analysis, the correlation between the presence of cardiovascular risk factors and thrombotic events. 49…”

“…This data confirm previous experiences showing, through retrospective analysis, the correlation between the presence of cardiovascular risk factors and thrombotic events. 49 The frequency of bleeding complications in patients with ET varies in different studies. In a 2012 international study conducted on 891 ET patients, 55 major bleeding events occurred (6.17%).…”

Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding

“…As discussed, due to the chronic manifestation of this disease, the main goal of ET management remains the prevention of fatal vascular complications which have been reported to be the leading cause of death [ 31 ]. Up to 24% of ET patients develop a vascular event before (13%) or after (11%) diagnosis with a lower rate in CALR -mutants as compared to JAK2 V617F/ MPL mutants and triple-negative cases [ 39 , 44 ]. Apart from having different molecular lesions and survival outcomes, JAK2 V617F-mutated ET patients had a higher hemoglobin level and WBC (the so-called “PV-like phenotype”), and a lower PC while a substantial fraction of CALR -mutants had a PC >1000 × 10 9 /L [ 39 ].…”

The Essential Thrombocythemia, Thrombotic Risk Stratification, and Cardiovascular Risk Factors

“…In 2017, a further enhancement of the IPSET-t was proposed by Tefferi and Barbui adding the negative effect of MPL mutation [9]. In an article entitled "Comparison between thrombotic risk scores in essential thrombocythemia and survival implications," we used the three available risk scores to classify the same monocentric group of patients, highlighting how many patients change thrombotic risk class when reclassified, although the high-risk group still remains the larger [10]. Today, the classification of thrombotic risk according to r-IPSET-t in patients with ET do not take into account CVR, and these conditions do not currently influence the choice of cytoreductive therapy in clinical practice, even if it could suggest to adopt a different dosage of acetylsalicylic acid or other antiplatelet drugs for thrombosis prophylaxis [11][12][13][14].…”