Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients

Abstract

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.

تقييم بعض انزيمات القلب والحديد في مصل مرضى بيتا-ثلاسيميا في مدينة ذي قار ، العراق

“…This means that an increase in the iron depot in β-thalassemia major patients leads to an increase in AST levels. Our current finding agreed with many previous studies 35,36,37 .…”

Korelasi Feritin dengan Sitokin Proinflamasi pada Pasien Talasemia Beta Di Kota Samarinda Kalimantan Timur

“…Hasil ini selaras dengan studi Fianza di RSUP Dr. Hasan Sadikin Bandung yang menyebutkan pasien talasemia paling banyak memiliki kadar feritin >2.500 ng/mL. 12 Tingginya kadar feritin pada subjek penelitian tidak terlepas akibat dari manifestasi proses transfusi yang berulang. Selain transfusi darah, kelebihan besi juga disebabkan faktor internal pada pasien talasemia yaitu proses eritropoesis tidak efektif, hemolisis kronis dan peningkatan penyerapan zat besi usus.…”

Therapeutic options and management approach on thalassemia an overview

“…Iron overload may have negative effects on the heart, kidneys, lungs, liver, and endocrine system, among other vital organs. (Fianza et al,2021). Previous studies substantiate that HbA2 is lower if iron deficiency or the 𝛼-thalassemia trait is present; it is unaffected by gender, smoking, or tribal allegiance.…”