Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients
Pandji Irani Fianza,
Anita Rahmawati,
Sri Hudaya Widihastha,
Shofura Afifah,
Mohammad Ghozali,
Andre Indrajaya,
Dilli Marayuzan Akbar Pratama,
Dimmy Prasetya,
Teddy Arnold Sihite,
Mas Rizky A. A. Syamsunarno,
Djatnika Setiabudi,
Suthat Fucharoen,
Ramdan Panigoro
Affiliations
Pandji Irani Fianza
Department of Internal Medicine, Division of Hematology and Medical Oncology, Faculty of Medicine, Universitas Padjadjaran/Hasan Sadikin General Hospital, Bandung, West Java, Indonesia
Anita Rahmawati
Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, Indonesia
Sri Hudaya Widihastha
Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, Indonesia
Shofura Afifah
Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, Indonesia
Mohammad Ghozali
Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, Indonesia
Andre Indrajaya
Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, Indonesia
Dilli Marayuzan Akbar Pratama
Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, Indonesia
Dimmy Prasetya
Department of Internal Medicine, Division of Hematology and Medical Oncology, Faculty of Medicine, Universitas Padjadjaran/Hasan Sadikin General Hospital, Bandung, West Java, Indonesia
Teddy Arnold Sihite
Department of Cardiology and Vascular Medicine, Faculty of Medicine, Universitas Padjadjaran/Hasan Sadikin General Hospital, Bandung, West Java, Indonesia
Mas Rizky A. A. Syamsunarno
Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, Indonesia
Djatnika Setiabudi
Department of Child Health, Faculty of Medicine, Universitas Padjadjaran/Hasan Sadikin General Hospital, Bandung, West Java, Indonesia
Suthat Fucharoen
Thalassemia Research Center, Institute of Molecular Biosciences, Mahidol University, Nakhonpathom, Thailand
Ramdan Panigoro
Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, Indonesia
Thalassemia is a genetic disease caused by disruption of globin chain synthesis leading to severe anemia and thus regular blood transfusion is necessary. However, there have been known transfusions-related consequences, including iron overload and multi-organ damage. The aims of this study were to evaluate liver and cardiac function in youth and adult transfusion-dependent Indonesian thalassemic patients and to assess its correlation with serum ferritin levels, as well as T2∗magnetic resonance imaging (MRI). Transfusion-dependent thalassemic (TDT) outpatients (n = 66; mean age, 21.5 ± 7.2 years) were carried out for the complete assessment consisting of blood test including liver enzyme and serum ferritin, followed by electrocardiography (ECG) and echocardiography. Subjects were also divided by serum ferritin levels into three groups: 5000 ng/mL. Additionally, subgroup analysis in patients with T2∗ MRI assessment was conducted. In terms of age of first blood transfusion, subjects with ferritin >5000 ng/mL were the youngest among others. The alanine aminotransferase (ALT) levels in group with serum ferritin >5000 ng/mL were significantly higher than those of the group with serum ferritin 5000 ng/mL had significantly lower tricuspid annular plane systolic excursion (TAPSE) when compared with those who had serum ferritin <2500 ng/mL. Similarly, TAPSE in patients with moderate cardiac siderosis based on cardiac T2∗ MRI was significantly lower than those without cardiac siderosis. There was significant, but only moderate correlation between serum ferritin and cardiac T2∗ MRI. Based on these findings, it is important to routinely monitor iron accumulation-related complications, including liver and cardiac damage in youth and adult TDT patients.