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 language | English |
|---|---|
| Pages (from-to) | 365-370 |
| Number of pages | 6 |
| Journal | Internal and Emergency Medicine |
| Volume | 14 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - 2019 |
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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 journal › Article
}
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 -