Risk factors for heart disease in transfusion-dependent thalassemia: serum ferritin revisited

on behalf of Webthal®, Giorgio Derchi, Carlo Dessì, Patrizio Bina, Maria Domenica Cappellini, Antonio Piga, Silverio Perrotta, Immacolata Tartaglione, Marianna Giuditta, Filomena Longo, Raffaella Origa, Antonella Quarta, Valeria Pinto, Gian Luca Forni

Research output: Contribution to journalArticle

Abstract

Heart disease remains a leading cause of morbidity and mortality in transfusion-dependent thalassemia (TDT), which can be attributed to several factors but primarily develops in the setting of iron overload. This was a retrospective cohort study utilizing Webthal® patient data from five major centers across Italy. Patients without heart disease were followed-up for 10 years (2000–2010) and data were collected for demographics, splenectomy status, serum ferritin and hemoglobin levels, and comorbidities associated with heart disease. Among 379 patients analyzed (mean age 22.9 ± 5.1 years, 47.8% men), 44 (cumulative incidence: 11.6%) developed heart disease during the period of observation. Splenectomy (p = 0.002) and serum ferritin level (p < 0.001) were the only risk factors with significant association with heart disease. A serum ferritin threshold of ≥ 3000 ng/mL was the best predictor for the development of heart disease (86.4% sensitivity and 92.8% specificity, AUC: 0.912, 95% CI 0.852–0.971, p < 0.001). On multivariate analysis, only a serum ferritin level ≥ 3000 ng/mL remained significantly and independently associated with increased risk of heart disease (HR: 44.85, 95% CI 18.85–106.74), with a 5- and 10-year heart disease-free survival of 58 and 39%. The association between iron overload and heart disease in patients with TDT is confirmed, yet a new serum ferritin level of 3000 ng/mL to flag increased risk is suggested.

Original languageEnglish
Pages (from-to)365-370
Number of pages6
JournalInternal and Emergency Medicine
Volume14
Issue number3
DOIs
Publication statusPublished - 2019

Fingerprint

Thalassemia
Ferritins
Heart Diseases
Serum
Iron Overload
Splenectomy
Italy
Disease-Free Survival
Area Under Curve
Comorbidity
Hemoglobins
Cohort Studies
Multivariate Analysis
Retrospective Studies
Observation
Demography
Morbidity
Sensitivity and Specificity
Mortality
Incidence

Keywords

  • Heart disease
  • Iron overload
  • Serum ferritin
  • Thalassemia

ASJC Scopus subject areas

  • Internal Medicine
  • Emergency Medicine

Cite this

Risk factors for heart disease in transfusion-dependent thalassemia : serum ferritin revisited. / on behalf of Webthal®; Derchi, Giorgio; Dessì, Carlo; Bina, Patrizio; Cappellini, Maria Domenica; Piga, Antonio; Perrotta, Silverio; Tartaglione, Immacolata; Giuditta, Marianna; Longo, Filomena; Origa, Raffaella; Quarta, Antonella; Pinto, Valeria; Forni, Gian Luca.

In: Internal and Emergency Medicine, Vol. 14, No. 3, 2019, p. 365-370.

Research output: Contribution to journalArticle

on behalf of Webthal®, Derchi, G, Dessì, C, Bina, P, Cappellini, MD, Piga, A, Perrotta, S, Tartaglione, I, Giuditta, M, Longo, F, Origa, R, Quarta, A, Pinto, V & Forni, GL 2019, 'Risk factors for heart disease in transfusion-dependent thalassemia: serum ferritin revisited', Internal and Emergency Medicine, vol. 14, no. 3, pp. 365-370. https://doi.org/10.1007/s11739-018-1890-2
on behalf of Webthal® ; Derchi, Giorgio ; Dessì, Carlo ; Bina, Patrizio ; Cappellini, Maria Domenica ; Piga, Antonio ; Perrotta, Silverio ; Tartaglione, Immacolata ; Giuditta, Marianna ; Longo, Filomena ; Origa, Raffaella ; Quarta, Antonella ; Pinto, Valeria ; Forni, Gian Luca. / Risk factors for heart disease in transfusion-dependent thalassemia : serum ferritin revisited. In: Internal and Emergency Medicine. 2019 ; Vol. 14, No. 3. pp. 365-370.
@article{573e3f372c974a51828b0d3102d0d413,
title = "Risk factors for heart disease in transfusion-dependent thalassemia: serum ferritin revisited",
abstract = "Heart disease remains a leading cause of morbidity and mortality in transfusion-dependent thalassemia (TDT), which can be attributed to several factors but primarily develops in the setting of iron overload. This was a retrospective cohort study utilizing Webthal{\circledR} patient data from five major centers across Italy. Patients without heart disease were followed-up for 10 years (2000–2010) and data were collected for demographics, splenectomy status, serum ferritin and hemoglobin levels, and comorbidities associated with heart disease. Among 379 patients analyzed (mean age 22.9 ± 5.1 years, 47.8{\%} men), 44 (cumulative incidence: 11.6{\%}) developed heart disease during the period of observation. Splenectomy (p = 0.002) and serum ferritin level (p < 0.001) were the only risk factors with significant association with heart disease. A serum ferritin threshold of ≥ 3000 ng/mL was the best predictor for the development of heart disease (86.4{\%} sensitivity and 92.8{\%} specificity, AUC: 0.912, 95{\%} CI 0.852–0.971, p < 0.001). On multivariate analysis, only a serum ferritin level ≥ 3000 ng/mL remained significantly and independently associated with increased risk of heart disease (HR: 44.85, 95{\%} CI 18.85–106.74), with a 5- and 10-year heart disease-free survival of 58 and 39{\%}. The association between iron overload and heart disease in patients with TDT is confirmed, yet a new serum ferritin level of 3000 ng/mL to flag increased risk is suggested.",
keywords = "Heart disease, Iron overload, Serum ferritin, Thalassemia",
author = "{on behalf of Webthal{\circledR}} and Giorgio Derchi and Carlo Dess{\`i} and Patrizio Bina and Cappellini, {Maria Domenica} and Antonio Piga and Silverio Perrotta and Immacolata Tartaglione and Marianna Giuditta and Filomena Longo and Raffaella Origa and Antonella Quarta and Valeria Pinto and Forni, {Gian Luca}",
year = "2019",
doi = "10.1007/s11739-018-1890-2",
language = "English",
volume = "14",
pages = "365--370",
journal = "Internal and Emergency Medicine",
issn = "1828-0447",
publisher = "Springer-Verlag Italia s.r.l.",
number = "3",

}

TY - JOUR

T1 - Risk factors for heart disease in transfusion-dependent thalassemia

T2 - serum ferritin revisited

AU - on behalf of Webthal®

AU - Derchi, Giorgio

AU - Dessì, Carlo

AU - Bina, Patrizio

AU - Cappellini, Maria Domenica

AU - Piga, Antonio

AU - Perrotta, Silverio

AU - Tartaglione, Immacolata

AU - Giuditta, Marianna

AU - Longo, Filomena

AU - Origa, Raffaella

AU - Quarta, Antonella

AU - Pinto, Valeria

AU - Forni, Gian Luca

PY - 2019

Y1 - 2019

N2 - Heart disease remains a leading cause of morbidity and mortality in transfusion-dependent thalassemia (TDT), which can be attributed to several factors but primarily develops in the setting of iron overload. This was a retrospective cohort study utilizing Webthal® patient data from five major centers across Italy. Patients without heart disease were followed-up for 10 years (2000–2010) and data were collected for demographics, splenectomy status, serum ferritin and hemoglobin levels, and comorbidities associated with heart disease. Among 379 patients analyzed (mean age 22.9 ± 5.1 years, 47.8% men), 44 (cumulative incidence: 11.6%) developed heart disease during the period of observation. Splenectomy (p = 0.002) and serum ferritin level (p < 0.001) were the only risk factors with significant association with heart disease. A serum ferritin threshold of ≥ 3000 ng/mL was the best predictor for the development of heart disease (86.4% sensitivity and 92.8% specificity, AUC: 0.912, 95% CI 0.852–0.971, p < 0.001). On multivariate analysis, only a serum ferritin level ≥ 3000 ng/mL remained significantly and independently associated with increased risk of heart disease (HR: 44.85, 95% CI 18.85–106.74), with a 5- and 10-year heart disease-free survival of 58 and 39%. The association between iron overload and heart disease in patients with TDT is confirmed, yet a new serum ferritin level of 3000 ng/mL to flag increased risk is suggested.

AB - Heart disease remains a leading cause of morbidity and mortality in transfusion-dependent thalassemia (TDT), which can be attributed to several factors but primarily develops in the setting of iron overload. This was a retrospective cohort study utilizing Webthal® patient data from five major centers across Italy. Patients without heart disease were followed-up for 10 years (2000–2010) and data were collected for demographics, splenectomy status, serum ferritin and hemoglobin levels, and comorbidities associated with heart disease. Among 379 patients analyzed (mean age 22.9 ± 5.1 years, 47.8% men), 44 (cumulative incidence: 11.6%) developed heart disease during the period of observation. Splenectomy (p = 0.002) and serum ferritin level (p < 0.001) were the only risk factors with significant association with heart disease. A serum ferritin threshold of ≥ 3000 ng/mL was the best predictor for the development of heart disease (86.4% sensitivity and 92.8% specificity, AUC: 0.912, 95% CI 0.852–0.971, p < 0.001). On multivariate analysis, only a serum ferritin level ≥ 3000 ng/mL remained significantly and independently associated with increased risk of heart disease (HR: 44.85, 95% CI 18.85–106.74), with a 5- and 10-year heart disease-free survival of 58 and 39%. The association between iron overload and heart disease in patients with TDT is confirmed, yet a new serum ferritin level of 3000 ng/mL to flag increased risk is suggested.

KW - Heart disease

KW - Iron overload

KW - Serum ferritin

KW - Thalassemia

UR - http://www.scopus.com/inward/record.url?scp=85048362979&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85048362979&partnerID=8YFLogxK

U2 - 10.1007/s11739-018-1890-2

DO - 10.1007/s11739-018-1890-2

M3 - Article

AN - SCOPUS:85048362979

VL - 14

SP - 365

EP - 370

JO - Internal and Emergency Medicine

JF - Internal and Emergency Medicine

SN - 1828-0447

IS - 3

ER -