Background and Aims: It has been proposed that iron overload in patients with β-thalassimia is associated with enhanced free-radical formation and/or defects in antioxidant defense. Iron facilitates the generation of free radical species (i.e. hydroxyl radical) via the Fenton reaction. Hydroxyl radicals are the main cause of tissue damage in biological systems. The aim of the present study was to examine the impact of iron overload in patients with β -thalassimia on plasma markers of oxidative stress including vitamin E and malonedialdehyde. Materials and Methods: Patients with major β -thalassimia and control subjects (age: 7.67±4.4.2 years and 10.20 Patient with major β -thalassimia 5.50; n=20 each) were recruited from Imam Hospital, Urmia. Plasma malonedialdehyde levels were analysed spectrophotometrically. Vitamin E concentrations were measured by HPLC coupled to spectrometric detector. Results: Plasma vitamin E levels were lower in patients with β -thalassimia than in control subjects (l4.07±4.25 µM vs 20.04±5.02 µM; p<0.005). Patients with β -thalassimia, on the other hand, had higher malonedialdehyde concentrations than in control subjects (0.34±0.03µM vs 0.19±0.04 µM; p<0.005). Both Iron and Ferritin levels were significantly elevated in patients with major β -thalassimia than in control subjects (160.97±45.54 µg/L vs 61.16±23.42 µg/L and 387.77±334.90 µg/L vs 50.29±34.15 µg/L, respectively). Discussion: This study has shown that oxidative stress resulting from iron overload in patients with β - thalassmia is linked with decreased antioxidant capacity and increased lipid peroxidation than in Control subjects.      

Introduction: β-THALASSEMIA, particularly the major form, is associated with significant morbidity, as it requires lifelong maintenance transfusion therapy to manage the condition. This transforms THALASSEMIA from a fatal childhood disease into a chronic disorder. Nonetheless, this therapeutic approach presents challenges due to its pathological adverse effects on cardiac health, including heart failure and arrhythmias. Discussion: Multiple lifelong transfusions, combined with the pathological effects of THALASSEMIA—such as hemolysis and ineffective erythropoiesis—exacerbate excessive iron deposition, primarily in the liver but most critically in the heart. This creates a vicious cycle between iron overload and cardiac dysfunction. Due to their high dependence on blood transfusions, THALASSEMIA major patients are predisposed to left-sided heart failure, resulting from both dilated and restrictive cardiomyopathy, as well as life-threatening arrhythmias and electrical disturbances. These complications arise from the heart’s overwhelmed capacity to clear free radicals. Cardiac dysfunction represents a critical complication requiring early detection and prompt intervention, underscoring the limitations of conventional echocardiography in diagnosing subclinical and systolic dysfunction—the latter often appearing only in advanced disease. Earlier risk stratification is essential, with recent studies highlighting the role of genetic predisposition, biomarkers, and advanced noninvasive imaging (MRI) in facilitating timely treatment initiation, such as iron-chelating therapy, to improve survival outcomes. Conclusions: Iron overload is an inevitable consequence for THALASSEMIA major patients requiring transfusions, as the human body lacks mechanisms to eliminate excess iron. These patients require careful observation, monitoring, and timely diagnosis according to standard guidelines to facilitate chelation therapy and prevent its harmful effects. This review examines the complex interplay between symptomatic management of THALASSEMIA, subsequent iron overload, and cardiac dysfunction in treated patients, with the goal of promoting early detection of therapeutic complications and timely intervention.

Introduction and aim: THALASSEMIA is the most prevalent hereditary anemia throughout the world. In Iran, there are about 25000 THALASSEMIA cases, and every year about 1500 new THALASSEMIA cases are born.This study aimed to determine prevalence of minor-B THALASSEMIA in Birjand.Material and methods: In this cross-sectional study, 480 students (260 males, 220 females) who were randomly selected and CBC was done for all of them.Then, microcytosis cases, after taking oral iron for a period of one month, were tested again; and those with unrelieved microcytosis were assigned to undergo hemoglobin A2 electrophoresis. Cases proved above 3. 7% were diagnosed and recorded as THALASSEMIA B cases.Results: From among 480 students, 34 (7.1%) suffered from microcytosis; seven of whom were excluded from the study because they didn't take oral iron. After taking oral iron for one-month period, microcytosis of 13 cases (2.7%) was relieved; from the remainder of 14 individuals, 5 cases (2 females and 3 males; 1% of the total) had HBA2 over 3.7%. (p=0.52).Conclusion: Considering the low prevalence of the mentioned gene in the region (1%) and the trivial success of the screening plan prior to marriage, it is recommended that screening plan be carried out at a different age level such as childhood or high school period.

Background and purpose: Previous studies indicate an important genetic factor in the etiology of β-THALASSEMIA and bipolar mood disorder. There have been several case reports implicating a possible association between the two conditions. But the results of a cross sectional study was not reconfirming. Regarding different patterns of mutations in different geographical areas, this study was performed to evaluate the genetic association between these two conditions in Mazandaran province in which β–THALASSEMIA is prevalent. Materials and methods: This case-control study was conducted on 110 patients with bipolar I disorder and 118 persons without any history of mood disorders in them and their family. Hemoglobin electrophoresis was performed in all cases and controls that showed microcytosis in their CBC. Hemoglobin A2 between 3.4 to 7 was considered for diagnosis of β-THALASSEMIA minor. Results: 8.18% of cases and 4.24% of controls were diagnosed to be heterzygote for β-THALASSEMIA, the difference was not statistically significant. Conclusion: The reported genetic relationship between Bipolar disorders and heterozygous β-THALASSEMIA probably has been a “false association”. However, as there are different mutations for minor THALASSEMIA in different populations, the authors are aware of low external validity of this study, and suggest farther studies on different populations.