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Background: Deferasirox (DFX) is a once-daily oral iron chelator with proven dose-dependent efficacy in patients with thalassemia major (TM). The reason for switching from DFX dispersible tablets (Exjade®) to DFX film-coated tablets (Jadenu®) was intolerance. Many patients also reported that deferasirox® did not taste good. In this study, we compared the effect of Jadenu® and Exjade® on satisfaction, convenience, cardiac/liver MRI T2*, serum ferritin levels, and biochemical profiles in patients with thalassemia major. Method: Sixty-two patients with thalassemia over 2 years of age, who had iron overload indicated by chelation therapy, were randomly divided into two groups. The first group (n = 32) is treated with Exjade®, and the second group (n = 30) is treated with Jadenu®. Laboratory investigations included alkaline phosphatase (ALK), alanine transferase (ALT), aspartate transferase (AST), and serum ferritin levels. Cardiac/liver MRI T2* levels and patient satisfaction and convenience, were assessed before and 1 year after starting therapy. Results: The study found that 53.3% of Jadenu® patients were satisfied with the taste of the medication compared to only 12.5% of Exjade® patients, which was statistically significant (p = 0.001). Additionally, 40% of Jadenu® patients were satisfied with the ease of taking the medication compared to 28.1% of Exjade® patients, and again, the difference was statistically significant (p = 0.047). A comparison of the cardiac MRI T2* levels between the two studied groups showed no significant difference (p = 0.851). Conclusion: Jadenu® offers patients an improved formulation that can be taken on an empty stomach, has a better taste, and presents fewer gastrointestinal tolerability concerns. Overall, patient satisfaction is higher with Jadenu®, which may improve adherence and reduce the frequency and severity of complications associated with iron overload. This, in turn, may help mitigate cardiovascular and hepatic complications from iron overload in the long term. Clinical Trial Registration: https://irct.behdasht.gov.ir/search/result?query=IRCT20210830052346N1 [ABSTRACT FROM AUTHOR]

Introduction: Beta-thalassemia is a prevalent autosomal recessive haemolytic disorder, particularly in the Middle East. Patients with beta-thalassemia major (βTM) require iron chelation therapy and regular blood transfusions, while those with beta-thalassemia intermedia (βTI) exhibit milder symptoms. This study aimed to evaluate cognitive function, behavioural problems, quality of life, and related aspects in individuals with βTM and βTI. Methods: The cross-sectional study included 20 βTM participants, 20 βTI participants, and 19 healthy controls. Participants underwent psychiatric interviews, the Wechsler Adult Intelligence Scale (WAIS-III), Symptom Checklist-90 Revised (SCL-90R), and Short-Form Health Survey (SF-36). Clinical data and laboratory investigations were also collected. Results: βTM participants exhibited lower vocabulary and picture completion scores on the WAIS-III compared to other groups. βTI participants had significantly higher scores on SCL-90R subscales for somatization, depression, anxiety, paranoid ideation, and psychoticism. Both patient groups reported poorer quality of life across all SF-36 domains compared with controls. Longer disease duration correlated with lower levels of somatization, interpersonal sensitivity, depression, anxiety, and paranoid ideation. Less frequent blood transfusions were associated with better physical and mental health summary scores but also higher somatization. Elevated serum iron levels corresponded with increased anxiety, hostility, and psychoticism symptoms, while higher serum ferritin related to more obsessive–compulsive behaviours but less somatization and psychoticism. Conclusions: Individuals with thalassemia exhibit cognitive deficits, psychological disturbances, and diminished quality of life, with distinct patterns depending on disease severity. Regular monitoring and appropriate interventions are crucial for optimizing their overall well-being and functioning. [ABSTRACT FROM AUTHOR]

Background: Patients with β-thalassemia major (TM) require lifelong blood transfusions, leading to iron overload, where active intervention is required. Aims and Objectives: The aim of the study was to evaluate real-world efficacy and safety of deferasirox (DFX) in TM patients at a tertiary care teaching hospital. Materials and Methods: A prospective observational study was conducted at thalassemia ward in a tertiary care teaching hospital in Gujarat for a period of 15 months from April 2021 to July 2022. A total of 100 patients were analyzed during the study period. Patient's demographic profile, laboratory investigation, and treatment details were analyzed using descriptive statistics. Statistical analysis was done through the statistical software GraphPad Prism 9.4.1 (681). Results: A total of 100 patients were included in the study. A serial significant decrease in median serum ferritin levels was observed in >13 years age group after initiation of DFX. A significant increase in the median dose of DFX was observed at 12 months in the age group of 2-≤13 years as compared to baseline (P < 0.05). Adverse reactions observed during the study were increased bilirubin level, increased alanine aminotransferase, and increased serum creatinine, rashes with itching, and arthralgia. Conclusion: A greater reduction in median serum ferritin levels was observed in the adult age group compared to pediatric patients. [ABSTRACT FROM AUTHOR]

Abstract Introduction Beta-thalassemia is a prevalent autosomal recessive haemolytic disorder, particularly in the Middle East. Patients with beta-thalassemia major (βTM) require iron chelation therapy and regular blood transfusions, while those with beta-thalassemia intermedia (βTI) exhibit milder symptoms. This study aimed to evaluate cognitive function, behavioural problems, quality of life, and related aspects in individuals with βTM and βTI. Methods The cross-sectional study included 20 βTM participants, 20 βTI participants, and 19 healthy controls. Participants underwent psychiatric interviews, the Wechsler Adult Intelligence Scale (WAIS-III), Symptom Checklist-90 Revised (SCL-90R), and Short-Form Health Survey (SF-36). Clinical data and laboratory investigations were also collected. Results βTM participants exhibited lower vocabulary and picture completion scores on the WAIS-III compared to other groups. βTI participants had significantly higher scores on SCL-90R subscales for somatization, depression, anxiety, paranoid ideation, and psychoticism. Both patient groups reported poorer quality of life across all SF-36 domains compared with controls. Longer disease duration correlated with lower levels of somatization, interpersonal sensitivity, depression, anxiety, and paranoid ideation. Less frequent blood transfusions were associated with better physical and mental health summary scores but also higher somatization. Elevated serum iron levels corresponded with increased anxiety, hostility, and psychoticism symptoms, while higher serum ferritin related to more obsessive–compulsive behaviours but less somatization and psychoticism. Conclusions Individuals with thalassemia exhibit cognitive deficits, psychological disturbances, and diminished quality of life, with distinct patterns depending on disease severity. Regular monitoring and appropriate interventions are crucial for optimizing their overall well-being and functioning.

OBJECTIVE: To identify the effectiveness and safety of thalidomide in transfusion-dependent beta Thalassemia Major (TDBTM) patients. METHODS: A comprehensive search of PubMed, Cochrane Library, and Embase was conducted between January 01, 2014, and April 17, 2024, using terms like “thalidomide”, “thalidomide”, “α- phthalimidoglutarimide”, “thalassemia”, “beta thalassemia”, “transfusion dependent thalassemia” using Boolean or wildcard operators. Studies published in English with an observational or experimental design, including more than 10 TDBTM patients treated with thalidomide for at least 3 months, were included. The review focused on patients of all ages and genders, evaluating the impact of thalidomide on transfusion requirements. The included trials, involved 780 participants (age range=1.5-27.2 years), showing improvements in hemoglobin, fetal hemoglobin (HbF), serum ferritin, spleen size, and quality of life. National Institute of Health tool was used for quality assessment. RESULTS: After screening 19462 records, 18147 remained after duplicates were removed. Of these, 18138 were excluded, leaving 09 studies for inclusion in the review. Conducted in Pakistan, China, India and Iraq (2014-2024), the studies included a single-arm trial, a double-blinded RCT, and an open-label RCT and Pre-post enrolling 780 participants. Thalidomide (50-150 mg/day) improved hemoglobin, HbF, serum ferritin, spleen size, and quality of life, with a mean follow-up of 13 months. Thalidomide therapy resulted in transfusion independence in 69.6 % (n= 543) patients. Adverse effects were reported in 41.2 % (n=322) patients. Study quality was rated as good. CONCLUSION: Thalidomide is a well-tolerated, safe, and effective treatment for TDBTM patients, but these findings require confirmation through well-designed clinical trials.

OBJECTIVE: To investigate the relationship between repeated blood transfusions and growth retardation in children with transfusion-dependent Thalassemia in Peshawar, Pakistan. METHODS: This cross-sectional study was conducted on transfusion-dependent thalassemia patients receiving treatment at the Fatimid Foundation, Peshawar Pakistan, between February and August 2022. Participants were categorized by age (2 years) to assess and measure growth patterns over six months. RESULTS: Out of 93 children with thalassemia major, 55 (59.1%) males and 38 (40.9%) females. Mean age of the participants was 10.86 ± 5.72 years, and mean age at their first transfusion was 8.13 ± 5.78 months. Mean body mass index was 16.38 ± 1.82 kg/m². Short stature was observed in 49 patients (52.7%), of whom 57.1% (n=28) were male and 42.9% (n=21) were female. Serum ferritin levels were significantly elevated in patients with short stature, with 57.1% (n=28/49) having ferritin levels >4001 μg/L compared to 40.9% (n=18/44) with normal stature. Only 3.2% of patients had normal ferritin levels. The ROC analysis identified a ferritin cut-off of 1636 μg/L for predicting short stature (sensitivity 86%, specificity 68%). Growth assessment showed that 71% of the children were

Background and Aim: Due to the adverse effects of their disease, patients with thalassemia major face many problems such as a lack of proper growth, enlarged spleen and liver, bone disorders, especially the bones of the head and face. As a result of the long treatment process of this disease, other aspects of their life are also affected. The physical and mental problems in this population of patients will lead to frustration and lower social performance and quality of life. The present study aimed to explore the effect of an educational intervention based on the PRECEDE model on quality of life of adults with thalassemia major in Hormozgan province. Methods: The present quasi‐experimental study was conducted on 234 adults with major thalassemia who visited the thalassemia medical centers in Hormozgan province in 2022. The participants were selected through a simple randomization from two cities of Hormozgan province (Bandar Abbas as the intervention group and Minab as the control). Each group had an equal number of participants (n = 117). The data were collected using a demographic information questionnaire, a researcher‐made questionnaire based on the model constructs and the SF‐12 quality of life questionnaire. The instruments were once administered before the intervention and once again 4 months after the intervention. The educational intervention included 3 sessions a week, each 60 min in length, shared in WhatsApp and Telegram. The data were analyzed in statistical package for the social sciences version 24 using Mann−Whitney U‐test, chi‐square test, Wilcoxon's test, and analysis of covariance. Findings: The results showed that after the educational intervention, the mean scores of knowledge, attitude, self‐efficacy, enabling afctors, reinforcing factors and the mean score of overall quality of life increased significantly in the intervention group compared to the control (p < 0.05). The covariance analysis showed when the effect of the pretest score is removed, the mean scores of the model constructs and the quality of life are significantly different in the two groups. Conclusion: Educational interventions based on the PRECEDE model can help identify factors affecting the quality of life on many aspects in patients with thalassemia major. Thus, these interventions help improve patients' quality of life. The present findings can guide health policy makers and experts use modern educational interventions to develop the educational content to promote a healthy lifestyle and self‐management in certain diseases such as thalassemia major. [ABSTRACT FROM AUTHOR]

Objectives: This multicenter, retrospective, population-based, matched-cohort study compared clinical characteristics and magnetic resonance imaging (MRI) findings, including hepatic, pancreatic, and cardiac iron levels and cardiac function, between 135 adult regularly transfused thalassemia intermedia (TI) patients (44.73 ± 12.16 years, 77 females) and 135 age- and sex-matched thalassemia major (TM) patients (43.35 ± 9.83 years, 77 females), enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network. Methods: The MRI protocol included the quantification of hepatic, pancreatic, and cardiac iron levels (R2* technique), the assessment of biventricular function parameters (cine images), and the detection of replacement myocardial fibrosis (late gadolinium enhancement technique). Results: Age, sex, frequency of splenectomy and chelation, and serum ferritin levels were not significantly different (p > 0.05) between the two groups, but TI patients started regular transfusions significantly later (p < 0.0001) and showed significantly lower pre-transfusion hemoglobin levels (p = 0.005). No difference was found in hepatic iron levels (p = 0.853). TI patients exhibited significantly lower pancreatic R2* values (p < 0.0001), also correcting for the duration of regular transfusions, and significantly lower cardiac R2* values (p < 0.0001). In the receiver operating characteristic analysis, pancreatic iron was the strongest discriminator between the two diseases. Left and right ventricular end-diastolic volume indexes were significantly higher in TI than in TM patients (p = 0.003 and p = 0.046, respectively), but the correction for the duration of regular transfusions removed the disease-specific differences (p > 0.05). Left ventricular (LV) mass index was significantly higher in TI (p = 0.049), while no difference (p > 0.05) was found in biventricular ejection fractions and replacement myocardial fibrosis. Conclusions: TI patients showed lower pancreatic and cardiac iron burden and more pronounced LV hypertrophy. These differences could not be explained by the different duration of the transfusional regimen. [ABSTRACT FROM AUTHOR]

The aim of this cross-sectional study was to investigate the relationship of left atrioventricular coupling index (LACI) and right atrioventricular coupling index (RACI) with demographics, clinical data, cardiovascular magnetic resonance findings, and cardiac complications (heart failure, arrhythmias, and pulmonary hypertension) in a cohort of patients with beta-thalassemia major (β-TM). We evaluated 292 β-TM patients (151 females, 36.72 ± 11.76 years) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia (E-MIOT) project. Moreover, we assessed 32 sex- and age-matched healthy controls (12 females, mean age 40.78 ± 14.35 years). LACI was determined by calculating the ratio of the left atrium end-diastolic volume to the left ventricle end-diastolic volume, while RACI was defined by calculating the ratio of the right atrium end-diastolic volume to the right ventricle end-diastolic volume. Compared to healthy control, β-TM demonstrated increased LACI (22.99 ± 13.58% vs. 16.05 ± 5.28%; p < 0.0001) and RACI (27.84 ± 10.30% vs. 17.06 ± 5.03%; p < 0.0001). Aging, diabetes, splenectomy, and the presence of late gadolinium enhancement (LGE) showed a significant positive association with both LACI and RACI. In stepwise regression analysis, the presence of LGE was found to be an independent predictor of both impaired LACI and RACI (β coefficient = 0.244, p < 0.0001 and β coefficient = 0.218, p = 0.003; respectively). LACI and RACI were not correlated with myocardial iron overload. Patients with cardiac complications had significantly higher LACI and RACI than patients without cardiac complications. In patients with β-TM, LACI and RACI were significantly associated with the presence of LV LGE. In addition, patients with cardiac complications had impaired LACI and RACI. [ABSTRACT FROM AUTHOR]

Background and Aim: Thalassemia major is a common genetic anemia disorder that causes dental problems such as dental caries and malocclusion. In this study, we investigated the effect of malocclusion severity on quality of life of patients with thalassemia major. Materials and Methods: In this descriptive-analytical cross-sectional study, 37 patients with thalassemia major participated. Patients' oral health quality of life was assessed based on orthognathic quality of life questionnaire (OQLQ) and malocclusion severity was measured by index of orthodontic treatment need (IOTN). Data was analyzed by Spss26 and One Way Anova with significance level less than 0.05. Results: In this study, it was found that quality of life associated with malocclusion was poor in 43.2% of patients with thalassemia major. Regarding the severity of malocclusion, 94.6% of people were in grade 3 to 5 and the highest prevalence was related to grade 3 (45.9%). There was no significant relationship between quality of life and malocclusion severity or gender (P>0.05). Conclusion: Approximately half of patients with thalassemia major had poor quality of life associated with malocclusion, and nearly all patients showed moderate to severe orthodontic treatment need. With increasing severity of malocclusion, no significant change was observed in quality of life. There was also no significant relationship between quality of life and gender and age. [ABSTRACT FROM AUTHOR]

BackgroundDeferasirox (DFX) is a once-daily oral iron chelator with proven dose-dependent efficacy in patients with thalassemia major (TM). The reason for switching from DFX dispersible tablets (Exjade®) to DFX film-coated tablets (Jadenu®) was intolerance. Many patients also reported that deferasirox® did not taste good. In this study, we compared the effect of Jadenu® and Exjade® on satisfaction, convenience, cardiac/liver MRI T2*, serum ferritin levels, and biochemical profiles in patients with thalassemia major.MethodSixty-two patients with thalassemia over 2 years of age, who had iron overload indicated by chelation therapy, were randomly divided into two groups. The first group (n = 32) is treated with Exjade®, and the second group (n = 30) is treated with Jadenu®. Laboratory investigations included alkaline phosphatase (ALK), alanine transferase (ALT), aspartate transferase (AST), and serum ferritin levels. Cardiac/liver MRI T2* levels and patient satisfaction and convenience, were assessed before and 1 year after starting therapy.ResultsThe study found that 53.3% of Jadenu® patients were satisfied with the taste of the medication compared to only 12.5% of Exjade® patients, which was statistically significant (p = 0.001). Additionally, 40% of Jadenu® patients were satisfied with the ease of taking the medication compared to 28.1% of Exjade® patients, and again, the difference was statistically significant (p = 0.047). A comparison of the cardiac MRI T2* levels between the two studied groups showed no significant difference (p = 0.851).ConclusionJadenu® offers patients an improved formulation that can be taken on an empty stomach, has a better taste, and presents fewer gastrointestinal tolerability concerns. Overall, patient satisfaction is higher with Jadenu®, which may improve adherence and reduce the frequency and severity of complications associated with iron overload. This, in turn, may help mitigate cardiovascular and hepatic complications from iron overload in the long term.Clinical Trial Registrationhttps://irct.behdasht.gov.ir/search/result?query=IRCT20210830052346N1

BACKGROUND: Despite prolonged life expectancy in patients with beta-thalassemia due to modern chelation therapy and planned blood transfusions, they still suffer from multisystem complications of this chronic hemolytic anemia, including endocrine system dysfunction. Under-recognized parathyroid hormone (PTH) dysfunction in these patients can manifest as bone pain and fractures. OBJECTIVE: The objective of the study was to evaluate PTH in patients with beta-thalassemia >10 years of age. METHODS: A cross-sectional study was performed on 76 randomly selected patients >10 years old with beta-thalassemia (including both thalassemia major and thalassemia intermedia) in the Al-Kut Hereditary Blood Disease Center in Wasit province, Iraq. The study covered the period from November 2021 to April 2022. Data were collected from patients’ files after written consent, including sex, age, ferritin level, mean hemoglobin level, type of chelation therapy, frequency of transfusion, mean calcium level, and whether the patients were splenectomized or not. RESULTS: Of the 76 patients enrolled in the study, 39 (51.3%) were males. There were 63 (82.9%) who had thalassemia major. The majority of cases (n = 55, 71%) were from the age group >15 years. Fifteen (19.7%) patients had low PTH levels. The age group >15 years had a higher frequency of having low PTH levels (P = 0.01 and Pearson factor - 0.29). The study found a positive correlation between low calcium and low PTH levels (P = 0.001, Pearson factor = 0.1). High ferritin levels had no significant correlation with PTH levels (P = 0.4). CONCLUSIONS: Beta-thalassemia patients can have a low PTH level. It can occur more often in patients >15 years old, which necessitates close monitoring for older thalassemia patients. A declining calcium level in these patients can correlate with a low PTH level.

BACKGROUND: Alloantibodies against donor red blood cells (RBCs) are developed by patients with transfusion-dependent thalassemia (TDT), which causes the donor RBCs to hemolyze. This decreases the transfusion’s efficacy and increases the risk of adverse effects like iron overload. MATERIALS AND METHODS: Two hundred and five TDT patients with an average age of 11 ± 6 years enrolled in this study underwent the Direct Coombs Test (DCT) to determine the frequency of alloimmunization. RESULTS: Most cases were of thalassemia major (TM) (76.09%), followed by thalassemia intermedia (TI) and (21.95%). Most of the cases were diagnosed and started on regular blood transfusion therapy between the ages of 1 and 10 years. Majority of the cases were born of parental consanguinity. Only 13.17% of the patients underwent splenectomy, of which TM cases were the majority. Even though O-positive was the most frequent blood type, most of the operated and alloimmunized cases belonged to the B-positive blood type. Only 10.24% of the patients had alloimmunization, with 51.85% of them developing it after splenectomy. Among all study participants, no correlation was found between the blood group and the type of thalassemia, alloimmunization, or splenectomy. Among TDT cases, there was a significant correlation (P < 0.0001) between alloimmunization and splenectomy. Alloimmunization and splenectomy correlated significantly with the total number of transfusions, the volume of blood transfused, and the transfusion initiation age. CONCLUSION: Our findings emphasize the significance of antigen typing in TDT patients before the first transfusion.

Thalassemia a common hereditary blood disorder resulting in anemia. It is an important public health problem, with a high prevalence in Southeast Asia and Mediterranean countries, and preventable through screening programs. However, due to its chronic nature, permanent physical changes, troublesome complications, and lifelong treatment, pediatric patients with thalassemia major are more prone to mental disorders and cognitive impairment. Internalizing and externalizing problems are higher in pediatric patients with thalassemia. Children with β-thalassemia major exhibit lower IQ scores than healthy children. Neurophysiology and neuroimaging examinations have shown abnormal results in children with thalassemia. Co-morbidity with mental disorders increases the mortality, morbidity, and total healthcare costs of patients with thalassemia. Therefore, routine evaluation of mental health problems is recommended to accommodate the early detection and prompt treatment of mental disorders. A multidisciplinary approach for thalassemia patients and families should be delivered by providing appropriate medical care, psychosocial support, and good transition care to improve survival and well-being, assist good social integration and daily functioning, and cope with the stress of chronic disease.

Non-adherence to treatment is a major worldwide concern for healthcare providers. This is especially important in chronic diseases where patients have to follow the prescribed treatment for a long time. Adherence to the treatment of patients with chronic diseases in developed countries is at an average of 50%, and this rate is lower in developing countries. Despite many studies on non-adherence to treatment, it has remained an unresolved issue for the past four decades. On the other hand, commonly used interventions and practices have not successfully improved adherence to prescribed treatments in patients with chronic diseases. Moreover, the available theories of adherence have little explanatory power to justify this issue.

Background: The immune response of thalassemia patients is different from usual; therefore, thalassemia patients are susceptible to infection. A study at Thalassemia Center in Cipto Mangunkusumo Hospital showed that all thalassemia patients experience zinc deficiency. The decreased cellular immune response is associated with zinc deficiency, whereas splenectomy exacerbates the condition. This study aims to evaluate the improvement of cellular immune response in splenectomized thalassemia major patients after zinc supplementation. Method: Randomized double-blinded controlled trial was conducted on splenectomized thalassemia major patients in 12 weeks period. The inclusion criteria were aged > 12 years and had negative HIV test results. The subject receiving corticosteroids were excluded. Fifty-six subjects were randomly divided into two groups, the zinc group and the placebo group. Between 2 groups, data on zinc serum, T lymphocyte count, CD4+ T lymphocyte count, CD8+ T lymphocyte count, and CD4+/CD8+ ratio were evaluated at the beginning and the end of the study and were analyzed with unpaired t-test, Mann Whitney test, and Wilcoxon Signed Rank test. Result: After zinc supplementation, only 18 of 28 subjects in the zinc group recovered normal zinc serum levels. There were no significant changes after zinc supplementation in all parameters of cellular immune response (p > 0.05) between the two groups. This might be due to the subjects' adherence, which was lower in the zinc group (75.82%) than in the placebo group (83.19%). Conclusion: The effect of zinc supplementation on cellular immune response in splenectomized thalassemia major patients had not been proven yet.

Abstract Background Thalassemia is one of the most common chronic diseases, which cause many problems for the patients, families, and health system. The aim of this study was to evaluate the effectiveness of the application of an educational program based on the Theory of Planned Behavior in adopting preventive behaviors from thalassemia. Methods This randomized controlled trial study was associated with the participation of 160 mothers of children suffering from thalassemia major, who were divided into two groups including intervention and control. Demographic information, knowledge, and data related to the constructs of the Theory of Planned Behavior were collected. Data were analyzed using SPSS16 software and descriptive and analytical tests. Results The mean scores of perceived behavioral control, behavioral intention, and behavior in the intervention group in the pre-intervention phase were 9.83 ± 1.45, 9.1 ± 21.32, and 2.1 ± 18.42, respectively. The stage after the intervention was increased to 12.00 ± 0.00, 11.51 ± 0.59, and 5 ± 0.37.95, respectively, and the difference of the means in the two stages was significant (P value 0.05). Conclusion The results of the study showed the effect of the training intervention based on the Theory of Planned Behavior on the promotion of preventive behaviors in mothers of children suffering from thalassemia major.

Background Aims: Hepcidin is low in β-thalassemia patients, suggesting a critical role in iron accumulation. Hepcidin has not been assessed in Jordanian thalassemia patients. There is variation in the response of superoxide dismutase (SOD) to iron overload and it has not been correlated with hepcidin. Thus, the aim of this study was to assess serum hepcidin and SOD in patients diagnosed with β-thalassemia intermedia and major, and to examine the correlation between hepcidin and SOD in those patients. Subjects and Methods: Sixty patients with β-thalassemia aged 2–25 years were recruited from the Thalassemia Unit of the Zarqa New Governmental Hospital. The patients were classified according to two forms of thalassemia and assessed for serum levels of hepcidin and SOD. Statistical analysis was performed to investigate the differences between the patients of the two forms, compare the levels of hepcidin and SOD with the normal reference ranges, and calculate their correlation coefficients. Results: The study sample included 31 thalassemia intermedia patients and 29 patients with thalassemia major who are 33 females and 27 males. All patients had significantly deficient hepcidin (2.21±0.16 ng/ml) and increased serum SOD (3.13±0.14 ng/ml) levels compared to normal values. Ferritin was very high (3036.9±309.7 ng/ml) in all patients and highest in adult patients and those with thalassemia major. No significant correlations were found between hepcidin or SOD and ferritin (p> 0.05). Only in child patients was hepcidin positively correlated with SOD (r=0.848, p=0.033). Conclusions: Jordanian β-thalassemia patients had hepcidin deficiency, contributing to iron accumulation. SOD had a significant protective role against oxidation in β-thalassemia. Chelation therapy is inadequate to treat iron overload, which is still the predominant contributor to health complications in thalassemia. Therefore, the use of hepcidin agonists could be a beneficial treatment of excess iron. [ABSTRACT FROM AUTHOR]

Thalassemia a common hereditary blood disorder resulting in anemia. It is an important public health problem, with a high prevalence in Southeast Asia and Mediterranean countries, and preventable through screening programs. However, due to its chronic nature, permanent physical changes, troublesome complications, and lifelong treatment, pediatric patients with thalassemia major are more prone to mental disorders and cognitive impairment. Internalizing and externalizing problems are higher in pediatric patients with thalassemia. Children with β-thalassemia major exhibit lower IQ scores than healthy children. Neurophysiology and neuroimaging examinations have shown abnormal results in children with thalassemia. Co-morbidity with mental disorders increases the mortality, morbidity, and total healthcare costs of patients with thalassemia. Therefore, routine evaluation of mental health problems is recommended to accommodate the early detection and prompt treatment of mental disorders. A multidisciplinary approach for thalassemia patients and families should be delivered by providing appropriate medical care, psychosocial support, and good transition care to improve survival and well-being, assist good social integration and daily functioning, and cope with the stress of chronic disease. [ABSTRACT FROM AUTHOR]

Introduction: This study aims to investigate the necessity of simultaneous cholecystectomy in thalassemia major patients undergoing splenectomy, in light of reports regarding the prevalence of gallstones in these patients and the overall incidence of the disease in Mazandaran. Methods: This study was conducted on all 247 patients with beta-thalassemia major, who underwent splenectomy surgery during 2010-2020 for any indication in medical centers affiliated to Babol University of Medical Sciences (without a history of this procedure). Their percentage was determined and a decision was made regarding simultaneous surgery of cholecystectomy and splenectomy. Sampling was done by census method and all cases were reviewed and patients were followed up by phone and the necessary information was extracted and recorded in the checklist. Data were described using SPSS 22 software and analyzed at a significance level of 0.05. Results: A total of 247 patients with an average age of 20.13 ± 3.98 years (minimum age 10 and maximum age 29) were included in the study. The frequency of simultaneous cholecystectomy was reported in 33 cases (13.4%). Out of 214 patients who did not need simultaneous cholecystectomy (57.9%), 183 patients underwent cholecystectomy after splenectomy (85.5%).In 28 people (11.3 percent) had normal ultrasound and 3 people (1.3 percent) died. The prevalence of gallstones was reported as 87.4 percent. It is The average time for cholecystectomy compared to splenectomy in 183 patients who underwent cholecystectomy was 13.41±6.94 months (minimum time interval 3 and maximum 28 months) and in the examination of ultrasound findings, 219 patients (88.7%) had stones. They had a gall bladder and normal ultrasound was reported in 28 people (11.3%). Conclusion: The study results indicate a high prevalence of gallstones in patients with thalassemia major. Therefore, it suggests that simultaneous cholecystectomy and splenectomy should be considered, even in asymptomatic cases. [ABSTRACT FROM AUTHOR]

Iron overload in beta transfusion-dependent thalassemia (β-TDT) may provoke oxidative stress and reduction of the antioxidant defenses, with serious consequences for the disease course and complications. The present study evaluated the oxidant/antioxidant status of β-TDT patients and its correlation with demographic, clinical, laboratory, and instrumental biomarkers. The OXY-adsorbent assay and the d-ROMs (Diacron, Grosseto, Italy) were evaluated in 58 β-TDT patients (mean age: 37.55 ± 7.83 years, 28 females) enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network. Iron overload was quantified with R2* magnetic resonance imaging. Mean OXY was 323.75 ± 113.19 μmol HClO/mL and 39 (67.2%) patients showed a decreased OXY-Adsorbent level (<350 μmol HClO/mL), of whom 22 (37.9%) showed severely reduced levels. Mean d-ROMs was 305.12 ± 62.19 UA; 12 (20.7%) patients showed oxidative stress, and 4 (6.9%) elevated oxidative stress. OXY showed a significant negative correlation with global and segmental cardiac iron levels. D-ROMs levels significantly correlated with markers of cardiovascular risk (aging, glycemia, and N-terminal pro-B-type natriuretic peptide). Antioxidant depletion is frequent in β-TDT patients, where OXY might serve as additive biomarker to assess heart iron status, whereas the d-ROMs might be helpful to assess the cardiovascular risk burden. [ABSTRACT FROM AUTHOR]

BACKGROUND: Alloantibodies against donor red blood cells (RBCs) are developed by patients with transfusion‑dependent thalassemia (TDT), which causes the donor RBCs to hemolyze. This decreases the transfusion’s efficacy and increases the risk of adverse effects like iron overload. MATERIALS AND METHODS: Two hundred and five TDT patients with an average age of 11 ± 6 years enrolled in this study underwent the Direct Coombs Test (DCT) to determine the frequency of alloimmunization. RESULTS: Most cases were of thalassemia major (TM) (76.09%), followed by thalassemia intermedia (TI) and (21.95%). Most of the cases were diagnosed and started on regular blood transfusion therapy between the ages of 1 and 10 years. Majority of the cases were born of parental consanguinity. Only 13.17% of the patients underwent splenectomy, of which TM cases were the majority. Even though O‑positive was the most frequent blood type, most of the operated and alloimmunized cases belonged to the B‑positive blood type. Only 10.24% of the patients had alloimmunization, with 51.85% of them developing it after splenectomy. Among all study participants, no correlation was found between the blood group and the type of thalassemia, alloimmunization, or splenectomy. Among TDT cases, there was a significant correlation (P < 0.0001) between alloimmunization and splenectomy. Alloimmunization and splenectomy correlated significantly with the total number of transfusions, the volume of blood transfused, and the transfusion initiation age. CONCLUSION: Our findings emphasize the significance of antigen typing in TDT patients before the first transfusion. [ABSTRACT FROM AUTHOR]

BACKGROUND: Despite prolonged life expectancy in patients with beta‑thalassemia due to modern chelation therapy and planned blood transfusions, they still suffer from multisystem complications of this chronic hemolytic anemia, including endocrine system dysfunction. Under‑recognized parathyroid hormone (PTH) dysfunction in these patients can manifest as bone pain and fractures. OBJECTIVE: The objective of the study was to evaluate PTH in patients with beta‑thalassemia >10 years of age. METHODS: A cross‑sectional study was performed on 76 randomly selected patients >10 years old with beta‑thalassemia (including both thalassemia major and thalassemia intermedia) in the Al‑Kut Hereditary Blood Disease Center in Wasit province, Iraq. The study covered the period from November 2021 to April 2022. Data were collected from patients’ files after written consent, including sex, age, ferritin level, mean hemoglobin level, type of chelation therapy, frequency of transfusion, mean calcium level, and whether the patients were splenectomized or not. RESULTS: Of the 76 patients enrolled in the study, 39 (51.3%) were males. There were 63 (82.9%) who had thalassemia major. The majority of cases (n = 55, 71%) were from the age group >15 years. Fifteen (19.7%) patients had low PTH levels. The age group >15 years had a higher frequency of having low PTH levels (P = 0.01 and Pearson factor ‑ 0.29). The study found a positive correlation between low calcium and low PTH levels (P = 0.001, Pearson factor = 0.1). High ferritin levels had no significant correlation with PTH levels (P = 0.4). CONCLUSIONS: Beta‑thalassemia patients can have a low PTH level. It can occur more often in patients >15 years old, which necessitates close monitoring for older thalassemia patients. A declining calcium level in these patients can correlate with a low PTH level. [ABSTRACT FROM AUTHOR]

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Abstract Background and Aim Due to the adverse effects of their disease, patients with thalassemia major face many problems such as a lack of proper growth, enlarged spleen and liver, bone disorders, especially the bones of the head and face. As a result of the long treatment process of this disease, other aspects of their life are also affected. The physical and mental problems in this population of patients will lead to frustration and lower social performance and quality of life. The present study aimed to explore the effect of an educational intervention based on the PRECEDE model on quality of life of adults with thalassemia major in Hormozgan province. Methods The present quasi‐experimental study was conducted on 234 adults with major thalassemia who visited the thalassemia medical centers in Hormozgan province in 2022. The participants were selected through a simple randomization from two cities of Hormozgan province (Bandar Abbas as the intervention group and Minab as the control). Each group had an equal number of participants (n = 117). The data were collected using a demographic information questionnaire, a researcher‐made questionnaire based on the model constructs and the SF‐12 quality of life questionnaire. The instruments were once administered before the intervention and once again 4 months after the intervention. The educational intervention included 3 sessions a week, each 60 min in length, shared in WhatsApp and Telegram. The data were analyzed in statistical package for the social sciences version 24 using Mann−Whitney U‐test, chi‐square test, Wilcoxon's test, and analysis of covariance. Findings The results showed that after the educational intervention, the mean scores of knowledge, attitude, self‐efficacy, enabling afctors, reinforcing factors and the mean score of overall quality of life increased significantly in the intervention group compared to the control (p

Background: Thalassemia major is a common type of genetic disorder found in improvement in the medical field, the life expectancy of these children was also increased so there arises a need to promote the quality of life of such patients. A lot of studies concentrate on various aspects of the life of thalassemia major, but no study was done to assess the physical fitness of such children which is quite important to regularize their exercise sessions. Objectives: The objective of this study was to find out the physical fitness of children with thalassemia patients. Materials and Methods: Nonexperimental study designed with 20 individuals diagnosed with thalassemia in major age groups between 7 and 18 both boys and girls. The outcome measure used in this study was 6-min walk test (6 MWT). Results: The mean distance covered in 6 min was 351.3 m on average. There was increase in heart rate and blood pressure after completion of the test, whereas oxygen saturation was found to drop post-6 MWT among these patients. Conclusion: Thalassemia major patients are found with this physical fitness when assessed with 6 MWT.

Thalassemia is an inherited blood disease which lacks hemoglobin, the protein that is carrying oxygen to the body. The severe one is called Thalassemia Major which needs special care about blood transfusion. The use of rule-based method to create an inference as the first diagnosis of Thalassemia Major is not effective as rules have to be achieved from long interview with the medical personnel. This research aims to create a model based on decision tree for first detection of Thalassemia Major. The dataset is obtained by interview of Thalassemia symptoms and primary data of medical records from a hospital. Classical decision tree models used are ID3, C4.5 and CART. The models are evaluated by Train-Test Split consists of 70% training and 30% testing data and k-Fold Validation for checking model’s overfitting or underfitting. The output of this research is a final tree model from the best performance of decision tree models. The final result shows that C4.5 has the best performance with accuracy 100% and not overfitting or underfitting. Also, C4.5 performs feature selections to its tree modeling to simplify the inference. In brief, decision tree based modeling is effective to be used as first detection of Thalassemia Major by interview symptoms with generating automatic rules from its tree model.

Background and purpose: Thalassemia is a common disorder in Iran and the world. The progress in the field of diagnosis and treatment of thalassemia made these people able to live for a longer period. In these patients, frequent blood transfusions are associated with numerous complications such as depression, anxiety, stress, etc. These problems are associated with a decrease in the quality of life. Therefore, this study was carried out to investigate and compare the quality of life in beta-thalassemia major patients with and without blood transfusion. Materials and methods: In this study, which was cross-sectional and descriptive-analytical in nature, a total of 170 patients with thalassemia major, aged 18-60, who were referred to the Thalassemia Center of Bo Ali Sina Hospital in Sari during the summer of 2014, were examined. The study was conducted with the ethical code IR.MAZUMS.REC.1402.18665 of Mazandaran University of Medical Sciences. According to the inclusion criteria, education level, and age range of the studied population, 116 patients out of 170 thalassemia major patients were studied. Considering that the number of thalassemia major patients with and without blood transfusions referring to the thalassemia center of Bo Ali Sina Hospital in Sari is almost equal, out of 116 patients with inclusion criteria, 58 patients are treated with periodic blood transfusions and 58 patients are under control without the need for transfusions. Blood was studied. Thalassemia patients are treated with blood transfusion every 15-30 days for injection and patients who do not need blood transfusion every 1-3 months to the thalassemia center for control. SF 36 standard questionnaire was used to check the quality of life. The reliability and validity of the Persian questionnaire were reported with Cronbach's alpha coefficient of 0.77 to 0.90 and vitality scale of 0.65. To analyze the variables, frequency indices, percentages, independent t-test, and chi-square test were used. Results: Among the thalassemia major patients studied, 52 (44.8%) were male and 64 (55.1%) were female. The average age in the group with and without blood transfusion was 34.75±8.35 and 39.33±8.18 years, respectively. There was no statistically significant difference in the average age between the two groups (P=0.128). There was no significant difference in the marital status, occupation, and level of education between the patients with and without thalassemia major injection. Among the investigated clinical complications, diabetes was significantly higher in the blood transfusion group (P=0.001). From the average coefficients of the eight indices of the SF 36 test, the subscale of general health (GH) was significantly better in patients with blood transfusion with a mean and standard deviation of 55.79±19.20 than in the non-injection group, 44.05±21.59 (P=0.002). Conclusion: It is suggested that in patients with thalassemia, especially thalassemia major, with or without blood transfusion, like other periodical control measures and medical treatment, evaluation of general health and quality of life should be carried out at regular intervals, to improve the quality of life of these patients, timely psychological or psychiatric interventions to be done at the required time.

Background: Pulmonary hypertension (PH) is associated with an increased risk of cardiac failure among asymptomatic patients with beta-thalassemia. This study aimed to determine the prevalence of PH and associated risk factors in patients with beta-thalassemia major (TM) and beta-thalassemia intermedia (TI) in Hormozgan province, Iran. Materials and Methods: Eighty-nine patients, including 65 TM and 24 TI, were enrolled in this cross-sectional study. The data regarding the gender, age, history of splenectomy, and type of chelating therapy were collected, and then hematocrit, hemoglobin (Hb), and serum ferritin levels were determined. Doppler echocardiography was performed for patients, and tricuspid regurgitation jet velocity (TRV)≥2.5 m/s was used to screen PH. Eventually, the data were analyzed using SPSS. Results: The prevalence of TRV≥2.5 m/s was 21.3% in all patients, indicating that it was not significantly different between patients with TM (21.8%) and TI (20.8%). There was a significant association between the prevalence of PH and the male gender. Based on the results, TRV≥2.5 m/s was observed in both children and adults. No significant association was detected between Hb, serum ferritin levels, and splenectomy with PH prevalence. Conclusion: The findings revealed that PH is common in both children and adult patients with TM and TI in our population and should be considered to choose the optimal treatment of patients with TM and TI.

Background Thalassemia patients are generally immunocompromised, making them susceptible to infection. A study at the Thalassemia Center at Dr. Cipto Mangunkusumo Hospital, Jakarta, showed that all thalassemia patients experienced zinc deficiency. Decreased cellular immune response has been associated with zinc deficiency, and splenectomy exacerbates the risk of infection. Objective To evaluate for improvement of cellular immune response in splenectomized thalassemia major patients after zinc supplementation. Methods This randomized, double-blind, controlled trial was conducted on splenectomized thalassemia major patients over a 12-week period. The inclusion criteria were splenectomized thalassemia major patients aged > 12 to 18 years with negative HIV test results. Patients receiving corticosteroids were excluded. Fifty-six subjects were randomly divided into two groups, the zinc group and the placebo group. Before and after the 12 -week treatment, we evaluated subjects' serum zinc, T lymphocyte count, CD4+ T lymphocyte count, CD8+ T lymphocyte count, and CD4+/CD8+ ratio and were analyzed with unpaired T-test, Mann Whitney test, and Wilcoxon Signed Rank test. Results After zinc supplementation, serum zinc serum levels in 18/28 subjects in the zinc group became normalized. None of the cellular immune response parameters were significantly different between the two groups after zinc supplementation (P > 0.05). This finding might have been due to the subjects' compliance which was lower in the zinc group (75.82%) than in the placebo group (83.19%). Conclusion Cellular immune response in splenectomized thalassemia major patients is not significantly changed after 12 weeks of zinc supplementation. [ABSTRACT FROM AUTHOR]

Purpose: Dual-energy X-ray absorptiometry (DXA) is the gold standard for measuring bone mineral density (BMD) with tolerable error rate, high precision, and excellent consistency. Our objective was to investigate the frequency and distribution of errors in a cohort of patients with Thalassemia major (TM). Methods: We reviewed the DXA examinations of 340 patients with β-TM followed by our institution, acquired in different imaging centers between 2009 and 2019. We collected sex and age at the time of the first examination and at the last visit, as well as BMD, T-score, and Z-score values. Errors were analyzed by anatomical site (lumbar spine, total hip, femoral neck). Results: Out of 5099 total DXA scans, 11.85% presented one or more errors. Specifically, the incorrect examinations were 315 out of 1707 (18.45%) at the lumbar spine level, 113 out of 1697 (6.66%) at the total hip, 176 out of 1695 (10.38%) at the femoral neck. Errors in vertebral inclusion were the most frequently registered (45.86%). A significant difference resulted from the comparison of the T-score and Z-score median values of all the lumbar spine DXA examinations and the correct ones (p value 0.037 and 0.0003, respectively). Conclusion: Although not directly involved in the performance and interpretation of DXA, physicians interested in osteoporosis management should be familiar with the protocols to minimize errors and allow the proper use of bone densitometry. DXA obtained at the spine level is more frequently affected by errors in patients with TM, potentially influencing the diagnostic assessment of bone health status. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to assess the impact of serum panel reactive antibodies (PRA) on the outcomes of allogeneic hematopoietic stem cell transplantation (HSCT) in pediatric thalassemia patients. Methods: A total of 73 pediatric patients with thalassemia were included in this single‐center study. Pre‐transplant PRA levels were evaluated, and the patients were divided into two groups: PRA‐negative (group 1; n = 44) and PRA‐positive (group 2; n = 29). Patient characteristics, including age, gender, donor type, stem cell source, and HLA compatibility, were analyzed. Transplant outcomes, including engraftment, transfusion requirements, and transplant‐related complications, were compared between the two groups. Further subgroup analysis was performed based on MFI values. Results: At the time of transplantation, patients in group 1 were younger than those in group 2 (p =.008). The number of fully matched donors within the family (MSD and MFD) was significantly higher in group 1 (p =.049). Additionally, Rh blood group incompatibility was higher in group 2 (p =.03). There was no statistically significant difference in the engraftment days of neutrophils, platelets, and erythrocytes between the two groups. The frequency of poor graft function and graft failure was higher in the group 2, but there was no statistically significant difference. Post‐transplant transfusion requirements for platelets and red blood cells were significantly higher in the group 2 (p <.001). Transplant‐related complications such as VOD, PRES, and aGvHD were more common in the group 2, but no statistical significance was detected. Conclusions: Serum PRA in pediatric thalassemia patients may impact the outcomes of HSCT. PRA‐positive patients had higher rates of blood product transfusion requirements. Although poor graft function, graft failure, and post‐transplant complications were more common in the group 2, statistical significance was not observed. Identifying patients with high PRA levels can assist in optimizing transplant strategies and post‐transplant care, leading to improved outcomes for the patients. [ABSTRACT FROM AUTHOR]

Background and purpose: Thalassemia is a common disorder in Iran and the world. The progress in the field of diagnosis and treatment of thalassemia made these people able to live for a longer period. In these patients, frequent blood transfusions are associated with numerous complications such as depression, anxiety, stress, etc. These problems are associated with a decrease in the quality of life. Therefore, this study was carried out to investigate and compare the quality of life in beta-thalassemia major patients with and without blood transfusion. Materials and methods: In this study, which was cross-sectional and descriptive-analytical in nature, a total of 170 patients with thalassemia major, aged 18-60, who were referred to the Thalassemia Center of Bo Ali Sina Hospital in Sari during the summer of 2014, were examined. The study was conducted with the ethical code IR.MAZUMS.REC.1402.18665 of Mazandaran University of Medical Sciences. According to the inclusion criteria, education level, and age range of the studied population, 116 patients out of 170 thalassemia major patients were studied. Considering that the number of thalassemia major patients with and without blood transfusions referring to the thalassemia center of Bo Ali Sina Hospital in Sari is almost equal, out of 116 patients with inclusion criteria, 58 patients are treated with periodic blood transfusions and 58 patients are under control without the need for transfusions. Blood was studied. Thalassemia patients are treated with blood transfusion every 15-30 days for injection and patients who do not need blood transfusion every 1-3 months to the thalassemia center for control. SF 36 standard questionnaire was used to check the quality of life. The reliability and validity of the Persian questionnaire were reported with Cronbach's alpha coefficient of 0.77 to 0.90 and vitality scale of 0.65. To analyze the variables, frequency indices, percentages, independent t-test, and chi-square test were used. Results: Among the thalassemia major patients studied, 52 (44.8%) were male and 64 (55.1%) were female. The average age in the group with and without blood transfusion was 34.75±8.35 and 39.33±8.18 years, respectively. There was no statistically significant difference in the average age between the two groups (P=0.128). There was no significant difference in the marital status, occupation, and level of education between the patients with and without thalassemia major injection. Among the investigated clinical complications, diabetes was significantly higher in the blood transfusion group (P=0.001). From the average coefficients of the eight indices of the SF 36 test, the subscale of general health (GH) was significantly better in patients with blood transfusion with a mean and standard deviation of 55.79±19.20 than in the non-injection group, 44.05±21.59 (P=0.002). Conclusion: It is suggested that in patients with thalassemia, especially thalassemia major, with or without blood transfusion, like other periodical control measures and medical treatment, evaluation of general health and quality of life should be carried out at regular intervals, to improve the quality of life of these patients, timely psychological or psychiatric interventions to be done at the required time. [ABSTRACT FROM AUTHOR]

Background: Thalassemia patients are at an increased risk of contracting opportunistic infections such as toxoplasmosis due to frequent blood transfusions and underlying immune deficiency. Objectives: This study aimed to determine the seroprevalence of Toxoplasma gondii among thalassemia patients compared to healthy controls and examine any relation with biochemical parameters like serum calcium and growth hormone levels. Methods: A total of 40 thalassemia anti-T. gondii IgG antibodies seropositive patient with 40 healthy subjects, were enrolled in the study. Serum analysis for anti- T. gondii IgG antibodies and growth hormone using ELISA. Genomic DNA extraction was performed on white blood cells and quantified by spectrophotometry. T. gondii infection was detected by real-time quantitative PCR (qPCR) based on amplification of a species-specific region of tachyzoite p30 gene using previously published primers. Serum levels of total calcium were estimated by colorimetric assay based on o-cresolphthalein complex method. ABO and Rh blood grouping, complete blood picture and other hematological analysis were performed based on standard methods. Results: Anti-T. gondii IgG antibodies were detected in 28% of thalassemia patients compared to only 5% of controls, indicating significantly higher exposure among thalassemia patients. Both serum calcium and growth hormones were also significantly lower among thalassemia patients. Further genomic analysis by qPCR revealed 72.5% of IgG positive thalassemia patients were positive for T. gondii DNA compared to 100% of IgG positive controls. Conclusion: The high susceptibility of thalassemia patients to T. gondii infection indicates the importance of screening donor blood to prevent transmission by frequent transfusions. Detailed investigations are warranted to elucidate the mechanisms behind disturbances in calcium homeostasis and growth hormone axis among thalassemia patients and any linkage with T. gondii co-infection. [ABSTRACT FROM AUTHOR]

Thalassemia is a blood disorder that results from decreased or lost synthesis of one or more globin chains. Thalassemia major as a red blood cell disorder requires high costs because it requires blood transfusions and the use of iron chelation drugs throughout the patient's life. This study aims to analyze the cost and effectiveness of oral iron chelation drugs such as deferasirox and deferiprone in patients with Thalassemia major at Setukpa Lemdiklat Polri Tk--II Hospital in 2023. Data was collected retrospectively using total sampling from patient medical records and hospital information system data. Based on the results of this study, the average total cost per treatment for thalassemia major patients who used deferasirox (IDR 52,969,775,-) was more expensive than deferiprone (IDR 49,407,613,-). The effectiveness of deferasirox (1,364 ng/mL) was bigger than that of deferiprone (1,210 ng/mL). The cost-effectiveness ratio of deferasirox (IDR 38,834,-) was lower than that of deferiprone (IDR 40,833,-). To change the drug from deferiprone to deferasirox requires an additional cost of IDR 23,130 per one additional unit. From the average costeffectiveness ratio, it can be concluded that deferasirox is more cost-effective than deferiprone. [ABSTRACT FROM AUTHOR]

Abstract Background Sepsis and thrombo-embolic disease are well known complications of thalassemia major. Intracardiac thrombi are however rare and can lead to diagnostic dilemmas. Case presentation We report the case of a 20-year-old female splenectomised thalassaemia major patient with severe iron overload, who presented with life threatening sepsis associated with a liver abscess. Discovery of a large oscillating intra cardiac lesion on 2D echocardiogram confirmed by Contrast Enhanced Computed Tomography (CECT) chest in the right atrium extending from the left hepatic vein through the inferior vena cava complicated the clinical course. After a prolonged Intensive Care Unit (ICU) stay supported with antibiotics and anticoagulation, she recovered with evidence of resolution of the intra cardiac thrombus. Conclusions Early recognition and prompt aggressive treatment of sepsis in patients with thalassemia is essential to prevent complications. Intracardiac thrombosis is a potentially treatable cause for an intra cardiac mass in patients with thalassemia major, which should not be missed.

The article presents a case study involving a 29-year-old male patient with thalassemia major who developed ventricular tachycardia and subsequent heart failure due to myocardial iron overload from frequent blood transfusions. It also discusses the importance of regular cardiac assessments using techniques like T2 sequence cardiac magnetic resonance imaging (CMR) and strain echocardiography to detect early signs of iron-induced cardiomyopathy,.

Background: Acute complications of transfusion are among leading causes of morbidity and mortality in chronically transfused patients. One of the main goals of the blood transfusion is reducing complications, improve blood safety and patient's health care worldwide. The purpose of present study was to investigate the prevalence of acute complications of transfusion in beta thalassemia major (TM) patients in Southeast Iran.Methods: In a prospective study, 23882 transfusions were evaluated in TM patients in thalassemia clinic for acute reactions during 2 years period. Data were collected by forms to record the information related to blood transfusion at the time of reaction occurence. Results: The assessment of the data revealed that 228 TM (0.95%) had acute complications of transfusion. Of 23882 transfusions in TM patients, 211 patients (0.87%) developed allergic reactions, 11 patients (0.04%) were diagnosed with febrile non-hemolytic reaction and 1 patient (0.004%) had experienced acute hemolytic transfusion reaction.Conclusions: The results of current study supports preventive measures for reduction of these complications to improve patient's health. Acute complications occurred may be due to insufficient ability to perform blood bank tests, lack of standard operating instructions and lack of necessary monitoring during the transfusion process. The best way to reduce the risk of blood transfusion reactions is providing special care to the patients during the first 24 hours after transfusion. Creating a consistent and continuous education system with standard operating instructions and establishing an error reporting system leads to improve knowledge about transfusion guidelines and best practices.

We evaluated the association of vitamin D and parathormone (PTH) levels with cardiac iron and function in beta-thalassemia major (β-TM) patients. Two-hundred and seventy-eight TM patients (39.04 ± 8.58 years, 56.8% females) underwent magnetic resonance imaging for the assessment of iron overload (T2* technique), biventricular function parameters (cine images), and replacement myocardial fibrosis (late gadolinium enhancement technique). Vitamin D levels were deficient (<20 ng/dL) in 107 (38.5%) patients, insufficient (20–30 ng/dL) in 96 (34.5%) patients, and sufficient (≥30 ng/dL) in 75 (27.0%) patients. Deficient vitamin D patients had a significantly higher frequency of myocardial iron overload (MIO; global heart T2* < 20 ms) than patients with sufficient and insufficient vitamin D levels and a significantly higher left ventricular end-diastolic volume index and mass index than patients with sufficient vitamin D levels. PTH was not associated with cardiac iron, function, or fibrosis. In the multivariate regression analysis, vitamin D, serum ferritin, and pancreatic iron levels were the strongest predictors of global heart T2* values. In receiver operating characteristic curve analysis, a vitamin D level ≤ 17.3 ng/dL predicted MIO with a sensitivity of 81.5% and a specificity of 75.3% (p < 0.0001). In TM, the periodic and regular assessment of vitamin D levels can be beneficial for the prevention of cardiac iron accumulation and subsequent overt dysfunction. [ABSTRACT FROM AUTHOR]

We evaluated pattern and clinical correlates of renal T2* measurements in adult β-thalassemia major (β-TM) patients. Ninety β-TM patients (48 females, 38.15 ± 7.94 years), consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia network, underwent T2* magnetic resonance imaging (MRI) for quantification of iron overload (IO) in kidneys, liver, pancreas, and heart. Ten (11.1%) patients showed renal IO (T2* < 31 ms). Global kidney T2* values did not show a correlation with gender, age, splenectomy, regular transfusions or chelation starting age, pre-transfusion hemoglobin, and serum ferritin levels. Global kidney T2* values showed an inverse correlation with MRI liver iron concentration (LIC) values (R = − 0.349; p = 0.001) and a positive correlation with global pancreas T2* values (R = 0.212; p = 0.045). Frequency of renal IO was significantly higher in patients with cardiac IO than in patients without cardiac IO (50.0% vs. 6.3%; p = 0.001). A significant inverse association was detected between global kidneys T2* values and lactate dehydrogenase (LDH) (R = − 0.529; p < 0.0001). In multivariate regression analysis, MRI LIC and LDH were the strongest predictors of global kidney T2* values. A MRI LIC > 4.83 mg/g dw predicted the presence of renal IO (sensitivity = 90.0%; specificity = 61.2%). Global kidney T2* values were inversely correlated with uric acid (R = − 0.269; p = 0.025). In conclusion, in adult β-TM patients, renal iron deposition is not common and is linked to both hemolysis and total body iron overload. [ABSTRACT FROM AUTHOR]

Introduction: The purpose of this study was to examine the effect of iron overload on the mobilization of peripheral blood stem cells (PBSCs) in pediatric patients with β-thalassemia major (TM). Methods: We retrospectively reviewed the records of 226 patients with TM from whom PBSCs were collected. Iron overload was based on serum ferritin level, and liver and cardiac iron overload was measured by magnetic resonance imaging (MRI) T2*. Results: The mean age of the TM patients was 7.35 ± 3.41 years. Of the patients, only 171 received MRI. Of the 171 patients, 35 had normal liver iron levels, 39 mild liver iron overload, 90 intermediate liver iron overload, and 7 severe liver iron overload. The intermediate + severe group was associated with significantly higher age and BMI and lower leukapheresis product white blood cell count and CD34+ cell levels (all, p < 0.05). Conclusion: Leukapheresis indices were similar between patients with different degrees of iron overload according to the ferritin level and cardiac iron overload, in which the later might be due to the small number of patients with cardiac overload. In patients with TM, the intermediate and severe liver iron overload was associated with poorer mobilization of PBSCs. [ABSTRACT FROM AUTHOR]

Background: Sepsis and thrombo-embolic disease are well known complications of thalassemia major. Intracardiac thrombi are however rare and can lead to diagnostic dilemmas. Case presentation: We report the case of a 20-year-old female splenectomised thalassaemia major patient with severe iron overload, who presented with life threatening sepsis associated with a liver abscess. Discovery of a large oscillating intra cardiac lesion on 2D echocardiogram confirmed by Contrast Enhanced Computed Tomography (CECT) chest in the right atrium extending from the left hepatic vein through the inferior vena cava complicated the clinical course. After a prolonged Intensive Care Unit (ICU) stay supported with antibiotics and anticoagulation, she recovered with evidence of resolution of the intra cardiac thrombus. Conclusions: Early recognition and prompt aggressive treatment of sepsis in patients with thalassemia is essential to prevent complications. Intracardiac thrombosis is a potentially treatable cause for an intra cardiac mass in patients with thalassemia major, which should not be missed. [ABSTRACT FROM AUTHOR]

Introduction: In Thalassemia major there is decrease or total suppression of hemoglobin polypeptide chain synthesis occurs. Patient require regular blood transfusion to maintain normal Hb level greater than 10 gm% [17]. An inevitable, important and potentially lethal complication of administering repeated blood transfusion to a child with thalassemia is gradual overloading of body with iron. Iron deposition on various organ affects their function including lungs. Pulmonary deposition of iron ultimately leads to decease in function of lungs which can be assessed by spirometry and their correlation can be derived. From the above facts one can apprehend the problem of associated complication in thalassemia major children receiving regular blood transfusion. Aim &Objectives: To determine the pulmonary function status in betathalassemia major patients receiving regular blood transfusion. To study the pattern of respiratory impairment using spirometry. Also to estimate iron overload status by estimating serum ferritin level and with these values correlation of respiratory impairment with iron overload in thalassemia patients. Methodology: Hospital based observational cross-sectional prospective study, of sample size 81 having diagnosed as Thalassemia major of patients between age group of 6year to 14 years. Just before transfusion, venous sample collected from all participants and serum ferritin levels were assessed. Serum ferritin levels were recorded in the chart of patients every 6 months. Serum ferritin was derived by calculating the average measurement over a 2-year period for each patient. Further patients were categorized into population group A and population group B whom having serum ferritin level greater than or equal to 2500ng/ml and less than 2500ng/ml respectively. PFT was performed on the day scheduled for blood transfusion. Results were expressed as a percentage of normal. To compare the clinical parameter and biochemical parameters, chi-square test of association has been used. For comparison of study variable, independent student t test was used. For correlation of number of blood transfusion with serum ferritin, pearson correlation co-efficient has been used. The results were compared by using SPSS software version 17. Results: Patients with higher number of transfusion i.e. greater or equal to 140 showed increased chance of pulmonary abnormality as evidenced by mean FEV1(91.82±3.556) which was significantly higher compared to patients with less number of transfusion (i.e. less than 140) was (86.23±2.224). Pulmonary function test parameter FEV1 when compared to serum ferritin level. It was found that mean FEV1(91.06±3.564) level of population with serum ferritin level less than 2500 is significantly higher than mean FEV1(81.18±4.177) of population with serum ferritin level higher or equal to 2500, which is statistically significant as evidenced by p-value of 0.001 Conclusion: Patients with higher number of transfusion showed increased chance of pulmonary abnormality. The severity of the restrictive disease increases in older age and with more transfusion iron burden, which is indicative of a central role of iron in the pathogenesis of pulmonary function abnormality, which is associated with thalassemia major. This study will emphasise, patients with Thalassemia major on regular blood transfusion need monitoring throughout treatment to avoid future Pulmonary complications. [ABSTRACT FROM AUTHOR]

Background: Given the association of hypomagnesemia with cardiac arrhythmia, the aim of this study was to investigate the relationship between serum magnesium levels with age and T2* magnetic resonance imaging (MRI) findings of the heart and liver in patients with thalassemia major (TM). Materials and Methods: In a descriptive cross-sectional study, a total of 62 patients with ß-thalassemia major aged 11-48 years were selected at the Amir-Kabir Hospital, Arak, Iran. Serum magnesium, ferritin, and iron levels of patients were measured, and the rate of cardiac and hepatic hemosiderosis of patients was extracted according to the routine T2*MRI method. Results: The mean age of the patients at diagnosis was 32.6 years. The comparison of TM patients with and without hepatic/cardiac hemosiderosis demonstrated that mean levels of serum ferritin, serum iron, and age were significantly higher in TM patients with cardiac hemosiderosis than in hepatic/cardiac non-hemosiderosis (P < 0.05); however, there was no significant difference in mean levels of serum magnesium in TM patients with and without hepatic/cardiac hemosiderosis (P = 0.279). Interestingly, the correlation of age with serum magnesium levels in TM patients revealed a statistically significant and moderate inverse correlation (r = -0.56, P = 0.013). Conclusion: Hypomagnesemia may occur in a time-dependent manner. It is recommended that, in addition to cardiac and hepatic T2*MRI, serum magnesium levels be measured by using magnesium replacement if necessary. [ABSTRACT FROM AUTHOR]

Thalassemia major is the most common chronic blood disease in the world, especially in Asia and Iran, and it gives rise to anxiety and reduces quality of life [QOL] in patients. The purpose of this study was to determine the effect of Benson relaxation technique on anxiety and QOL in patients with thalassemia major. This semi-experimental clinical trial study was conducted on 140 patients with thalassemia major in two intervention groups [n = 70] and control group [n = 70] in Ali Asghar Hospital, Zahedan.The data were collected using a demographic information form, the Spielberger State-Trait Anxiety Inventory [STAI], and the World Health Organization Quality of Life-BREF [WHOQOL-BREF] and self-report checklist. The control group received routine care, whereas the intervention group, we first completed the questionnaires; then, Benson relaxation technique was taught to each patient in three one-hour sessions in the presence of a family member for three consecutive days. Finally, they were asked to practice this technique twice a day for 12 weeks. The questionnaires were completed again one and three months after the intervention. Data analysis showed no significant difference between the mean scores of anxiety and QOL and its dimensions in the two groups at baseline [P > 0.001]. One and three months after the relaxation technique, however, the intervention group experienced a statistically significant difference in the mean scores of anxiety and QOL and its dimensions [P < 0.001]. The results confirmed that Benson Relaxation Technique reduces anxiety and improves the QOL of patients with thalassemia major. Clinical Trials Registration: IRCT20200926048842N2. [ABSTRACT FROM AUTHOR]

Objective To evaluate the safety and efficacy of the collection of peripheral blood stem cells (PBSCs) in pediatric patients with thalassemia major (TM) weighing 20 kg or less. Methods PBSCs collection data of 170 pediatric patients with TM weighing 20 kg or less from January 2013 to December 2020 in our center were reviewed. Safety was assessed by the occurrence of adverse events during apheresis procedures, and efficacy was evaluated by the number of CD34+ cells collected. Results A total of 171 PBSCs procedures were performed on 170 patients with TM weighing 20 kg or less, with a median age of (4.98±1.53) years and a median weight of (17.30±2.18) kg. The probability of collecting at least 1×106 CD34+ cells/kg during a single course of apheresis was 99.41% (169/170), with a median (5.88±4.23) ×106 CD34+ cells collected per kg of weight of the recipient. A minimum pre-apheresis hemoglobin (Hb) of 60 g/L in patients with TM weighing 20 kg or less was safe and feasible. The most common adverse event of G-CSF mobilization in TM patients is bone pain, with the incidence of 7.65% (13/170), which was higher than that of healthy children donors in our center. The most common adverse events during the collection were pain at the puncture site of the femoral vein (6.47%, 11/170) and low pressure of the fluid (2.92%, 5/170). And no serious complications related to PBSCs mobilization, central venous catheter(CVC)placement or the apheresis procedure occurred. Conclusion PBSCs collection by COM.TEC blood cell separator in children weighing 20 kg or less is safe and efficacious.

Introduction: Cardiac complications are the leading cause of death in thalassemia patients. It is assumed that progressive iron accumulation results in myocyte damage. Myocardial T2* measurement by cardiac MRI quantifies iron overload. We aimed to study the association between left and right ventricular (LV and RV) function and iron deposition estimation by cardiac MRI T2* in a sample of Iranian patients. Methods: Cardiac MRI exams of 118 transfusion-dependent thalassemia major patients were evaluated retrospectively. Biventricular function and volume and myocardial and liver T2* values were measured. The demographic and lab data were registered. Poisson and chi-square regression analyses investigated the correlation between the T2* value and ventricular dysfunction. Results: The study participants’ mean (SD) age was 32.7y (9.02), and 47.46% were female. Forty-nine cases (41.52%) revealed at least uni-ventricular dysfunction. LV dysfunction was noted in 20 cases, whereas 47 patients revealed RV dysfunction. The risk of LV dysfunction was 5.3-fold higher in patients with cardiac T2* value less than 10msec (RR=5.3, 95% CI=1.6, 17.1, P

Thalassemia is an inherited hematologic disease in which the body makes an abnormal form of hemoglobin. Patients are diagnosed with thalassemia major due to severe anemia and clinical symptoms. Thalassemia major is a common disease in the Turkish Republic of Northern Cyprus (TRNC) and other Mediterranean countries. Thalassemia major is a chronic and severe health problem. Thalassemia major damages organs due to intense iron accumulation and deep anemia. It negatively affects the quality of life and shortens life. Also, due to chronic disease, patients experience psychological and social problems. In this review, we present thalassemia major in the TRNC. The rate of patients has decreased with prevention programs and no sick baby has been born after 2001 in the TRNC. However, existing thalassemia patients have many physical and psychosocial problems and the treatment processes the patients are challenging. Since TRNC is an island that receives immigration, it has a sociological texture that includes different cultures, which can increase the dimensions of this problem. Therefore, this review focuses on the thalassemia process and the psychosocial problems caused by the disease in the TRNC.