Risk factors for heart disease in transfusion-dependent thalassemia: serum ferritin revisited
Giorgio Derchi1, Carlo Dessì2, Patrizio Bina2
1Unità di Cardiologia, Ospedale Galliera, Genoa, Italy.
Insights
Heart disease is a major risk in transfusion-dependent thalassemia (TDT) due to iron overload. A serum ferritin level of 3000 ng/mL or higher significantly predicts heart disease development in TDT patients.
Area of Science:
- Cardiology
- Hematology
- Internal Medicine
Background:
- Heart disease is a primary cause of mortality in transfusion-dependent thalassemia (TDT).
- Iron overload is a major contributing factor to cardiac complications in TDT patients.
Purpose of the Study:
- To identify risk factors for heart disease in TDT patients.
- To determine a specific serum ferritin threshold for predicting heart disease risk.
Main Methods:
- Retrospective cohort study using Webthal® patient data from five Italian centers (2000-2010).
- Analysis of 379 TDT patients, collecting data on demographics, splenectomy, serum ferritin, and hemoglobin levels.
- Statistical analysis including multivariate analysis to identify independent risk factors.
Main Results:
- 44 out of 379 patients (11.6%) developed heart disease.
- Splenectomy and elevated serum ferritin levels were significant risk factors.
- A serum ferritin threshold of ≥3000 ng/mL demonstrated high sensitivity (86.4%) and specificity (92.8%) for predicting heart disease.
- Serum ferritin ≥3000 ng/mL was an independent predictor (HR: 44.85) of heart disease risk.
Conclusions:
- Confirms the strong association between iron overload and heart disease in TDT.
- Suggests a serum ferritin level of 3000 ng/mL as a critical threshold for increased heart disease risk in TDT patients.
- Highlights the importance of monitoring and managing iron overload to prevent cardiac complications.
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.
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