Blood transfusion therapy for β‐thalassemia major and hemoglobin E β‐thalassemia: Adequacy, trends, and determinants in Sri Lanka

Background Regular blood transfusion therapy still remains the cornerstone in the management of β‐thalassemia. Although recommendations are clear for patients with β‐thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E β‐thalassemia. In this study, we aim to describe the adequacy, trends, and determinants of blood transfusion therapy in a large cohort of pediatric patients with β‐thalassemia major and hemoglobin E β‐thalassemia. Methods/procedure This cross‐sectional study was performed among all regularly transfused patents with β‐thalassemia aged 2 to 18 years attending three large thalassemia centers in Sri Lanka. Data were collected using an interviewer‐administered questionnaire, perusal of clinical records, and physical examination of patients by trained doctors. Results A total of 328 patients (male 47%) were recruited; 83% had β‐thalassemia major, whereas 16% had hemoglobin E β‐thalassemia. Sixty‐one percent of patients had low pretransfusion hemoglobin levels ( 200 mL/kg/year) by a majority (56%). Median pretransfusion hemoglobin was significantly lower in patients with hemoglobin E β‐thalassemia compared with β‐thalassemia major (P