Do Transfusion Dependent E-Βeta Thalassemics Behave Differently from Patients with Βeta-Thalassemia Major?

OBJECTIVE: To compare the blood transfusion pattern between children with transfusion dependent Eβ-thalassemia and β-thalassemia major.
METHODS: 168 children (age 3 months to 12 years) with transfusion dependent Eβ thalassemia and β thalassemia major were admitted to the hospital. 120 children who met our inclusion criteria, were selected and detailed history including various parameters indicating the blood transfusion pattern were recorded.
RESULTS: In this study 72 children (60%) of the patients were transfusion dependent Eβ thalassemia. They started receiving blood transfusion (BT) at a later age(p < 0.0001), they received BT less frequently(p = 0.001), the total number of blood transfusions received up to 5 years of age were less in number(p < 0.0001), the pre-transfusion Hb levels were higher (p < 0.0001) and the peak ferritin level was much lower in them (p < 0.0001). Their transfusion requirement was much less, need for splenectomy was less (p < 0.006), their spleen span and liver span were also less than the children with β-thalassemia major.
CONCLUSION: Our study clearly depicts that transfusion dependent Eβ thalassemia which is more common in our region shows a wide variation from β thalassemia major patients with respect to various parameters including their transfusion pattern. © Indian Society of Hematology and Blood Transfusion 2021.