Hematologic profiles of Beta thalassemia major patients: An institutional cross-sectional study

Abstract

Introduction: Thalassemia is inherited blood disorder characterized by decreased hemoglobin with two main types-alpha and beta thalassemia. There are two main varieties of β thalassaemia, β0 thalassaemia, in which no β globin is produced, and β+ thalassaemia, in which some β globin is produced, but less than normal. As the patient is diagnosed, regular blood transfusions and chelating treatment is given lifelong. In this study, we observed the haematological profiles of multi transfused thalassemia major patients with the accompanying complications. Aims and Objectives: To study the clinical features with RBC parameters, serum ferritin levels, chelation therapy and prevalence of blood transfusion related infection in multi transfused beta thalassemia major patients. Materials and Method: In this cross sectional study, total 90 diagnosed thalassemia major patients were taken with their complete history, physical examination and laboratory parameters such as-complete blood counts. All the patients were monitored for serum ferritin levels and chelation therapy along with screening for transfusion transmitted infections over a year. All the data were compiled and appropriate statistical analysis was done. Results: In present study out of 90 patients, 54 patients were males and 36 patients were females. Mean age of 15.26 ± 7.04 years with 68.9% (N=62) of cases required twice monthly transfusion, 30.0% (n=27) cases required once a month transfusion. 25 cases were found to be HCV+ (27.78%), 3 were HIV + (3.3%) and 62 cases were n on-reactive (68.89%). On first post transfusion CBC we found that 30 cases (33.33%) were found to be adequately transfused with levels > 10 gm%, where as in second results 34 cases (37.78%) were found to be adequately transfused with levels >10 gm%. Mean of average yearly average ferritin values in the study duration was found to be 4724 ± 287.65 ng/mL. The chelating agent dose was with mean 1040 mg and standard deviation 476.19. Conclusion: In the present study, it is concluded that haematological parameters like Haematocrit, Red Blood Cell mass, Haemoglobin and Mean Corpuscular volume had large covariances and Serum Ferritin levels was positively correlated with chelation therapy along with increased rate of transfusion transmitted infections in multi transfused thalassemia major patients.