Iron overload in beta thalassemia major patients

Abstract

Aims: Beta thalassemia is the most common monogenic hereditary hemoglobin disorder, which poses a major health burden to SriLanka. Regular transfusions of erythrocytes required for survival of these patients lead to inevitable iron overload, which is manifested, by elevated serum ferritin levels. Progressive deposition of iron leads to dysfunction and failure of the major organs. The aim of this study was to evaluate the iron overload of the beta thalassemia major patients in one of the thalassemia centres in Sri Lanka and to find its effect on growth status of the patients. Methods: The study included forty patients with confirmed diagnosis of beta thalassemia major, undergoing any chelation treatment. The mean age of the study group was 10.97±5.9 years with a range of 2-20 years. The patients were interviewed for the socio-demographic variables and their medical histories were obtained from the hospital files. Serum ferritin concentration, height and weight of the patients were measured and body mass index (BMI) was calculated. Results: The mean serum ferritin concentration was 2992.2±1575.35 ng/ml which showed a significant correlation with age and duration of blood transfusion. The mean z-score for height was -2.3±1.06 and 50% of the patients were stunted. The mean z-score for BMI was -1.32±1.28 and 35% of the patients were wasted. Both height and BMI had no significant correlation with iron overload of the patients. Conclusion: Iron overload and growth retardation were common in beta thalassemia major patients of the treatment center evaluated in this study in Sri Lanka. However, there was no significant relationship between physical growth and iron overload.

Body iron status of children and adolescents with transfusion dependent β-thalassaemia: trends of serum ferritin and associations of optimal body iron control

“…The mean serum ferritin level of our patient cohort was 1778 µg/l which is considerably lower than the value (2992 µg/l) reported in a recent study done among forty patients in another centre in Sri Lanka [ 16 ]. Similarly, two recent studies done in India [ 17 ] and Pakistan [ 18 ] reported very high mean serum ferritin values, 2767 and 4236 µg/l, respectively among patients with thalassaemia.…”

Cytokine Dependent Hematopoietic Cell Linker (CLNK) is Highly Elevated in Blood Transfusion Dependent Beta-Thalassemia Major Patients

“…28 A positive association between duration of blood transfusion and degree of body iron was observed previously. 29 Furthermore, some studies reported positive correlations between serum ferritin and duration of transfusion and age of the patients. 29 Moreover, the interaction between CLNK and iron overload biomarkers may be explained by the formation of microvesicles in RBCs.…”

“…29 Furthermore, some studies reported positive correlations between serum ferritin and duration of transfusion and age of the patients. 29 Moreover, the interaction between CLNK and iron overload biomarkers may be explained by the formation of microvesicles in RBCs. Bailey et al (2007) 7 found that the cyclic adenosine monophosphate (cAMP)-dependent pathway plays a role in γ-globin gene (HBG) expression in adult erythroblasts in β-thalassaemia.…”

Long non-coding RNAs MALAT1, MIAT and ANRIL gene expression profiles in beta-thalassemia patients: a cross-sectional analysis

“…The pretransfusion hemoglobin level was done on cell Dyne-3700 fully automated cell counter (Abbott Diagnostics, Wiesbaden, Germany). The pretransfusion levels over one year period were traced from the past medical records of the patients and the average was considered as the mean pretransfusion hemoglobin level [29]. The diagnosis of β-thal was confirmed by high-performance liquid exchange chromatography (HPLC) using Hemoglobin A1c/A2/F BIO-RAD D-10 dual program on D-10 analyzer (Bio-Rad, Gladesville, Australia).…”