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 -