Haematophysiological Study of Transfusion Dependent Beta-thalassaemia Patients in a Tertiary Care Hospital of Odisha, India

Abstract

Introduction: The Transfusion Dependent Beta-thalassaemia (TDT) is an autosomal recessive disorder that affects the red blood cells, both in the decreased as well as absence in production of Adult Haemoglobin (HbA) and characterised by severe anaemia, splenomegaly and bone deformities and require lifelong transfusion therapy, iron chelation and/ or bone marrow transplantation for successful control. Aim: To evaluate the haematophysiological status of various TDT patients. Materials and Methods: This is a cross-sectional study conducted in the Hematology Department of SCB Medical College and Hospital Cuttack, Odisha, India. The study was done in the duration of 10 months, from April 2021 to January 2022. The blood samples were collected from 62 diagnosed cases of Beta-thalassaemia. Haematophysiological parameters were studied by asking research questionnaire developed for this purpose and analysing various clinical complaints and laboratory data. The haemoglobin variants were analysed by fully automated capillary zone electrophoresis to confirm the diagnosis of homozygous state of Beta-thalassaemia by decreased or absence of globin chain synthesis (HbA2) of the haemoglobin component. The data was anlysed using Statistical Package for Social Sciences (SPSS) version 19. Results: In the present study majority of the patients 39 (62.9%) were below the age of 10 years. The clinical examination showed anaemia in all the patients with haepatomegaly and splenomegaly in 51 (82.25%) and 57 (91.93%) cases respectively. The mean haemoglobin was markedly decreased to 5.98±1.69 ranged from 3 gm% to 10 gm% followed by Foetal Haemoglobin (HbF) was raised up to 78.39±26.73% (normal absent to 0.02%) and adult haemoglobin (HbA2) was increased up to 8.00±5.72 (normal 95% to 98%). Conclusion: TDT is one of the commonest haemoglobinopathy causing major health problem. Common sign and symptoms includes low-grade fever, severe anaemia and haepatosplenomegaly. The Present study helped the families having TDT beta thalassaemia for early detection and avail the facilities given by Government of Odisha.

Keywords

• anaemia
• haemoglobin variants
• beta-globin gene