Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients

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 e...

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Main Authors: 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
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:Anemia
Online Access:http://dx.doi.org/10.1155/2021/5581831
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author 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
author_facet 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
author_sort Pandji Irani Fianza
collection DOAJ
description 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: < 2500 ng/mL, 2500–5000 ng/mL, and >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 <2500 ng/mL. Additionally, youth and adult TDT patients whose 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.
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spelling doaj-art-ced201394581436ab4b0ab25f8a759752025-02-03T05:49:17ZengWileyAnemia2090-12672090-12752021-01-01202110.1155/2021/55818315581831Iron Overload in Transfusion-Dependent Indonesian Thalassemic PatientsPandji Irani Fianza0Anita Rahmawati1Sri Hudaya Widihastha2Shofura Afifah3Mohammad Ghozali4Andre Indrajaya5Dilli Marayuzan Akbar Pratama6Dimmy Prasetya7Teddy Arnold Sihite8Mas Rizky A. A. Syamsunarno9Djatnika Setiabudi10Suthat Fucharoen11Ramdan Panigoro12Department of Internal Medicine, Division of Hematology and Medical Oncology, Faculty of Medicine, Universitas Padjadjaran/Hasan Sadikin General Hospital, Bandung, West Java, IndonesiaResearch Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, IndonesiaResearch Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, IndonesiaResearch Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, IndonesiaResearch Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, IndonesiaResearch Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, IndonesiaResearch Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, IndonesiaDepartment of Internal Medicine, Division of Hematology and Medical Oncology, Faculty of Medicine, Universitas Padjadjaran/Hasan Sadikin General Hospital, Bandung, West Java, IndonesiaDepartment of Cardiology and Vascular Medicine, Faculty of Medicine, Universitas Padjadjaran/Hasan Sadikin General Hospital, Bandung, West Java, IndonesiaResearch Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, IndonesiaDepartment of Child Health, Faculty of Medicine, Universitas Padjadjaran/Hasan Sadikin General Hospital, Bandung, West Java, IndonesiaThalassemia Research Center, Institute of Molecular Biosciences, Mahidol University, Nakhonpathom, ThailandResearch Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, IndonesiaThalassemia 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: < 2500 ng/mL, 2500–5000 ng/mL, and >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 <2500 ng/mL. Additionally, youth and adult TDT patients whose 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.http://dx.doi.org/10.1155/2021/5581831
spellingShingle 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
Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients
Anemia
title Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients
title_full Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients
title_fullStr Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients
title_full_unstemmed Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients
title_short Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients
title_sort iron overload in transfusion dependent indonesian thalassemic patients
url http://dx.doi.org/10.1155/2021/5581831
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