XAS and RR Structural Analysis of Hemoglobin and EPR Spectroscopic Labelling of Red Blood Cell Membranes Isolated from Thalassemia Patients in Jakarta, Indonesia

A study on thalassemia intermedia and major patients in Jakarta was initiated to obtain a comprehensive picture of metabolic dysregulation, iron overload, oxidative stress, and cell damage. Data were collected from a group of 10 transfusion-dependent patients in an age range of 11-25 years and another group of 5 frequently transfused (for at least 15 years) patients aged 17-30 years. A third group comprises 5 patients (aged 7 to 14 years) who had not yet obtained transfusions. The 10 controls were voluntary students without diagnosis or clinical signs of thalassemia up to 30 years of age. The study was approved by the Ethical Clearance Board of the Medical Faculty and all blood samples from controls and patients were obtained on fully informed consent. Levels of antioxidants (vitamins A, C, E and B-carotene) and reactive thiols are considerably decreased in transfused patients, whereas signs of iron overload and cell damage are increased (serum iron, ferritin, transferrin saturation, SGOT, SGPT, y-GT, bilirubin)