Introduction: Iron overload is a common feature of thalassemia intermedia due to regular blood transfusion and increased gastrointestinal iron absorption. Early detection and adequate iron chelator can decrease morbidity and mortality from iron overload. Liver iron concentration (LIC) by MRI T2* is the best non-invasive way to measure body iron stores. However, this method is expensive and not available nationwide in Indonesia. The aim of this study was to identify liver iron overload and correlation of transferrin saturation, serum ferritin, liver MRI T2* and LIC with transient liver elastography in adult thalassemia intermedia patients. Methods: This is a cross-sectional study of 45 patients with thalassemia intermedia with blood transfusion and with and without iron chelator therapy. The study was conducted at Cipto Mangunkusumo Hospital from August through October 2016. We performed measurements of transferrin saturation, serum ferritin level, transient liver elastography and liver MRI T2*. Pearson and Spearman correlation tests were used to evaluate the correlation between transient liver elastography with transferrin saturation, serum ferritin, liver MRI T2*and LIC. Results and Discussion: This study showed that 64.4% of study subjects are β-Hb E thalassemia intermedia. Furthermore, 84.4% of study subjects have regular transfusion. Based on liver MRI T2*all subjects suffered from liver iron overload, 48.9% had severe degree. Median value of liver MRI T2* was 1.6 ms. Mean serum ferritin was 2831 ng/mL, with median transferrin saturation of 66%. Mean of LIC corresponding to liver MRI T2* and mean liver stiffness measurement was 15.36± 7.37 mg Fe/gr dry weight and 7.7± 3.8 kPa, respectively. Liver stiffness correlated with serum ferritin (r=0.651; p=0.000), liver MRI T2* (r=− 0.357; p=0.016), and LIC (r=0.433; p=0.003). No correlation was found between liver elastography and transferrin saturation (r=0.204; p=0.178). Conclusion: Serum ferritin, liver MRI T2*and LIC correlated with liver elastography. No correlation was found between transferrin saturation and liver elastography.

Background: Iron overload is a major problem in patients with transfusion-dependent beta-thalassemia (TDT). Reports on the correlation between iron overload and endocrine function with growth retardation in such a population in Indonesia have not been established. Therefore, this study aims to obtain a profile of iron load and endocrine function of adult transfusion dependent beta-thalassemia patients and their correlation with growth retardation. Methods: A cross-sectional study was performed, involving adult homozygous and HbE beta-thalassemia patients receiving blood transfusions at the Cipto Mangunkusumo Hospital, Jakarta. Iron overload was represented by serum ferritin (FS) and transferrin saturation (TS), while the endocrine function was examined by the Thyroid Stimulating Hormone-sensitive (TSHs), free T4 (fT4), and insulin-like growth factor-1 (IGF-1). The results were analyzed using bivariate analysis plus Pearson and Spearman correlation tests. Results: In general, 58 subjects were selected from 224 adult transfusion dependent beta- thalassemia patients, consisting of 31 males (53.4%) and 27 females (46.6%). Furthermore, their median age was 21 (18– 24) years, while the subclinical hypothyroid proportion was 32.7% and low IGF-1 levels were detected in 79.3% of the total population. There was a weak negative correlation between FS and fT4 (Spearman rho=− 0.361; p=0.003), as well as IGF-1 (Spearman rho=− 0.313; p=0.008), but FS and TSHs had no correlation (Spearman rho=0.074; p=0.29). Also, there was no correlation between ST with TSHs (Spearman rho=0.003; p=0.492), fT4 (Spearman rho=0.018; p=0.448), and IGF-1 (Spearman rho=− 0.142; p=0.143). Conclusion: Based on serum ferritin, iron overload is discovered to have a negative correlation with free T4 and insulin-like growth factor-1.

Background: Iron overload is a major problem in both transfusion-dependent (TDT) and non-transfusion-dependent thalassemia (NTDT). It has been known to increase oxidative stress. Meanwhile, blood transfusion as main therapy for thalassemia increases iron overload. One of the markers of oxidative stress is malondialdehyde (MDA). This study aims to provide data on MDA levels in adult thalassemia patients, and to compare the levels before and after transfusion in patients with TDT and NTDT. Methods: This is a cross-sectional, pre-post study in adult patients with thalassemia major and intermedia that received blood transfusion with or without iron-chelating agents in Cipto Mangunkusumo Hospital. Blood samples were taken immediately before the transfusion and one day after. Serum ferritin (SF) assays were conducted by electrochemiluminescence immunoassay method, while transferrin saturation (TS) was calculated by dividing serum iron by the binding capacity. Subsequently, plasma MDA levels assays were performed using the Wills method, and data analysis was conducted using the t-test/Mann–Whitney and Pearson/Spearman correlation test, depending on the data distribution. Results: The 63 respondents recruited consist of 51 TDT and 12 NTDT patients, and their median plasma MDA level before and after transfusion was 0.49 μmol/L and 0.45 μmol/L, respectively. Before transfusion, there was no correlation between SF and MDA, and TS and MDA levels. After the transfusion, there was no correlation between, SF and MDA, or TS and MDA levels. Conclusion: There is no significant difference in MDA levels before and after transfusion. Although blood transfusion increases the iron load in thalassemia patients, there was no increase in median MDA level after transfusion. Meanwhile, there was no correlation between markers of iron overload and MDA level in thalassemia patients both before and after transfusion.

Introduction: The achievement of blood transfusion hemoglobin targets in transfusion-dependent beta-thalassemia patients is influenced by several factors such as genotype, hypersplenism, blood compatibility, donor blood adequacy, and transfusion interval. Failure to achieve these targets leads to an increase in the size of the spleen. Meanwhile, the post-transfusion hemoglobin of thalassemia patients that is not regularly evaluated has made it difficult to determine donor adequacy. Therefore, this study aims to determine the proportion of patients who achieve optimal pre- and post-transfusion hemoglobin levels, determine the factors involved, and the relationship between achieving hemoglobin levels with spleen enlargement in adult transfusion-dependent beta-thalassemia patients. Methods: This retrospective cohort study was conducted using total sampling of adult thalassemia transfusion-dependent patients at Cipto Mangunkusumo Hospital. Data were obtained through medical records. Results: A hundred and ten study subjects fulfilled inclusion criteria. The results showed that the blood transfusion deficit < 30 mL/kg/year was associated with achieving pre- and post-transfusion hemoglobin targets (p = 0.008). Furthermore, there were significant differences between the groups that achieved the pre- and post-transfusion target hemoglobin levels on the reduction of spleen enlargement in centimeters (p < 0.001). However, thalassemia genotype, blood compatibility, and transfusion interval did not correlate with the achievement of pre- and post-transfusion hemoglobin. Conclusion: The achievement of pre- and post-transfusion hemoglobin levels in adult transfusion-dependent beta-thalassemia patients significantly reduced spleen enlargement and contributed to better patient outcomes.

Background: The use of regular blood transfusions and iron chelation therapy to treat thalassemia has improved survival and increased the incidence of osteoporosis. Moreover, iron toxicity is one of the contributing factors that reduce bone mass density in adult transfusion-dependent beta-thalassemia patients. Therefore, this study aims to determine the proportion of low bone mass density in adult thalassemia patients and transferrin saturation, as well as serum ferritin, which correlates to the skeletal condition. Methods: This is a cross-sectional study conducted in Thalassemia and Hematology Medical Oncology Clinics of Cipto Mangunkusumo Hospital in March 2016. The anthropometric data and hemoglobin levels were obtained before transfusion. Subsequently, the average ferritin levels, bone mineral density, and radiographic results were obtained. Results: The percentage of adult thalassemia major and intermedia patients with low bone mass density was 68%. Also, there was a weak inverse correlation between bone mass density and transferrin saturation (r = − 0.329, p = 0.01), while no correlation was shown between bone mass density and ferritin (r = − 0.088, p = 0.504). The transferrin saturation cutoff point value used to distinguish the incidence of low and normal bone density in patients with transfusion-dependent beta-thalassemia was 89.5%. In addition, there was weak correlation between Singh index and bone mass density (r = 0.273, p = 0.038). Conclusion: Among the transfusion-dependent beta-thalassemia patients, 68% had low bone mass density, which inversely correlated to transferrin saturation. Furthermore, the cutoff value of transferrin saturation to differentiate the incidence of low and normal bone density in thalassemia major compared to thalassemia intermedia was 89.5%. Singh Index correlates weakly with bone mass density and might be used to detect low bone mass density in remote healthcare facilities.

Background: People living with transfusion dependent thalassemia have a high risk of becoming infected with COVID-19. This can be caused by both internal factors, namely the formation of alloantibodies and autoimmune disorder, and external factors such as routine visits for blood transfusions. Chronic complications of thalassemia also render them more vulnerable to infectious diseases, including COVID-19. However, anecdotal data shows that thalassemia patients experience less incidence of COVID-19 compared to the general population. Purpose: This study aims to find the correlation between COVID-19 in thalassemia-dependent transfusion patients in Indonesia and Southeast Asia. Patients and Methods: This study used a cross-sectional design. The study was conducted at the Division of Hematology and Medical Oncology of the Cipto Mangunkusumo Hospital in Jakarta from May 2020–August 2021. The total sampling method was used involving all thalassemia major patients who had been infected with COVID-19 (obtained directly from medical record and through the thalassemia patients-parents foundation). Results: From 10,397 patients with thalassemia, 67 (0.64%) people were infected by COVID-19 and 2 (2.9%) were deceased. Meanwhile, the incidence of COVID-19 in the general population of Indonesia was 0.87% (more than in the thalassemia population). This means that thalassemia might provide additional protection against COVID-19 due to several mechanisms. This phenomenon has also been seen in other countries with a high prevalence of thalassemia, wherein there are less COVID-19 cases despite the pandemic. On the contrary, countries with low rates of thalassemia had experienced deadly surges of the pandemic. Conclusion: Indonesia and other countries with a high prevalence of thalassemia have lower COVID-19 incidence than countries with low prevalence of thalassemia. Thalassemia might provide additional protection against COVID-19. Well-designed studies are needed to provide better evidence on the protective effect of thalassemia on COVID-19.