Predictors of survival and cause of death in patients with essential thrombocythemia

Leković, Danijela; Gotić, Mirjana; Šefer, Dragan; Ajtić, Olivera Mitrović; Čokić, Vladan P.; Milić, Nataša

doi:10.1111/ejh.12517

Cited by 11 publications

(4 citation statements)

References 38 publications

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“…The overall mortality was 13.17%, which is in agreement with previous reports which delineate a lower average survival in men diagnosed with ET (14 years versus 20 years) [13][14][15]. In our investigation, ET patients who were associated with hypertension had a three-fold higher risk of mortality (RR-3.11 95% CI (1.05-9.15); p = 0.05).…”

Section: Discussionsupporting

confidence: 92%

Essential Thrombocythemia: One-Center Data in a Changing Disease

Pirciulescu

Găman²,

Mihailescu³

et al. 2022

Medicina

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Introduction: Essential thrombocythemia is a chronic myeloproliferative neoplasm associated with thrombo-hemorrhagic events and the progression to myelofibrosis or acute myeloid leukemia. The purpose of this article is to present real-world data on ET cases diagnosed and managed between 1998 and 2020 in the largest, tertiary hematology reference center in Romania and to evaluate the impact of thrombotic events on survival. Methods: A real-world, retrospective cohort-type study was conducted. We collected and statistically analyzed data from 168 patients who met the 2016 WHO diagnostic criteria for ET and who were managed between 1998 and 2020 in our center. Results: The median age at diagnosis of ET was 51.8 years, with a female predominance (66.07%). The JAK2V617F mutation was detected in 60.71% of patients. Leukocytosis at diagnosis was associated with a higher risk of thrombosis, and JAK2V617F-positive cases exhibited a 1.5-fold higher risk of developing thrombotic events. The average survival in ET with major thrombosis was 14.5 years versus 20.6 years in ET cases without major thrombosis. Other predictors of survival were high-risk IPSET score and age >60 years. Conclusions: Romanian patients diagnosed with ET are generally younger than 60 years and are predominantly female. The occurrence of thrombotic events was influenced by gender, leukocyte count at diagnosis and JAK2V617F positivity. Survival was impacted by age, the presence of JAK2V617F mutation, hypertension, major thrombotic complications and IPSET score. Notably, these findings warrant careful interpretation and further confirmation in the setting of prospective studies.

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Section: Discussionsupporting

confidence: 92%

Essential Thrombocythemia: One-Center Data in a Changing Disease

Pirciulescu

Găman²,

Mihailescu³

et al. 2022

Medicina

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“…These risk factors, along with advanced age, have since been incorporated into an international prognostic score for ET (IPSET); the particular three‐tiered prognostic model assigned a score of 2 points for age ≥60 years and one point each for leukocyte count ≥11 x 10(9)/L and thrombosis history: low risk (no risk factors; median survival not reached), intermediate risk (1–2 points; median survival ̴ 25 years) and high risk (3–4 points; median survival ̴ 14 years). Current literature lists several other, reportedly independent, risk factors for survival in ET: prefibrotic morphology, anemia, cardiovascular risk factors, diabetes, tobacco use, and male sex . The latter association was further underlined by recent epidemiological data that suggested longer survival in women versus men with ET .…”

Section: Introductionmentioning

confidence: 99%

Gender and survival in essential thrombocythemia: A two‐center study of 1,494 patients

et al. 2017

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Based on suggestive information from recent epidemiologic data and earlier retrospective studies, we revisited the effect of gender on survival in 1,494 patients with essential thrombocythemia (ET). The primary study population included 904 patients from the Mayo Clinic (median age 58 years; 65% females); risk distribution, according to the international prognostic score for ET (IPSET), was 23% high, 42% intermediate and 35% low. Multivariable analysis that included IPSET-relevant risk factors identified male sex (HR 1.6, 95% CI 1.3-2.0), age ≥60 years (HR 4.3, 95% CI 3.4-5.4) and leukocyte count ≥11 × 10(9)/L (HR 1.5, 95% CI 1.3-1.9) as independent predictors of shortened survival. These findings were confirmed by analysis of a separate cohort of 590 ET patients (65% females) from the University of Florence, Italy, with corresponding HRs (95% CI) of 1.6 (1.1-2.5), 4.6 (2.2-9.5) and 1.8 (1.1-2.8). The independent prognostic effect of gender was further corroborated by a separate multivariable analysis against IPSET risk categories; HR (95% CI) for the Mayo Clinic/Florence cohorts were 1.5/1.6 (1.2/1.1-1.8/2.5) for male sex, 6.8/7.5 (5.0/3.1-9.3/18.3) for IPSET high risk and 2.8/4.1 (2.1/1.8-3.8/9.5) for IPSET intermediate risk. Furthermore, the survival disadvantage in men was most apparent in IPSET high risk category and in patients older than 60 years. In both patient cohorts, thrombosis history garnered significance in univariate, but not in multivariable analysis. The observations from the current study suggest that women with ET live longer than their male counterparts and that gender might supersede thrombosis history as a risk variable for overall survival.

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“…In a recent study on 826 Mayo Clinic patients with ET, PV, or PMF, the respective median survivals were approximately 20 years for ET, 14 years for PV, and 6 years for Lekovic et al published a study on 244 consecutive ET cases with a median follow-up of 7 years that revealed increased risk of death between patients with CV risk factors. 8 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 applied three different thrombotic risk models for ET available in clinical practice (conventional, IPSET-t, current stratification) to 191 ET patients followed in our hematology unit, over a period of 24 years, from 1994 to 2018.…”

Section: Introductionmentioning

confidence: 99%

“…Cardiovascular comorbidities as arterial hypertension, diabetes, hyperlipidemia, and exposition to cigarette smoke frequently encountered at the diagnosis of ET and represent the main cause of death. Lekovic et al published a study on 244 consecutive ET cases with a median follow‐up of 7 years that revealed increased risk of death between patients with CV risk factors …”

Section: Introductionmentioning

confidence: 99%

Comparison between thrombotic risk scores in essential thrombocythemia and survival implications

Santoro

Accurso

Mancuso

et al. 2019

Hematological Oncology

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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.

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Predictors of survival and cause of death in patients with essential thrombocythemia

Abstract: The addition of CV risk factors allows better prognostic assessment by delineating the intermediate-risk category and improved identification of the high-risk patients.

Cited by 11 publications

References 38 publications

Essential Thrombocythemia: One-Center Data in a Changing Disease

Essential Thrombocythemia: One-Center Data in a Changing Disease

Gender and survival in essential thrombocythemia: A two‐center study of 1,494 patients

Comparison between thrombotic risk scores in essential thrombocythemia and survival implications

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