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( < 0.0001), they received BT less frequently( = 0.001), the total number of blood transfusions received up to 5 years of age were less in number( < 0.0001), the pre-transfusion Hb levels were higher ( < 0.0001) and the peak ferritin level was much lower in them ( < 0.0001). Their transfusion requirement was much less, need for splenectomy was less ( < 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.