Background: β-thalassaemia major is one of the chronic hemolytic anemias resulting from defect in β-globin chain. It requires frequent blood transfusion plus other treatment modalities. These treatment modalities may be associated with certain immunologic modulations.
Objective: To assess the immunity status in children with β-thalassaemia major under different treatment regimens within El Minia, Egypt.
Subjects and Methods: One hundred forty-four children were enrolled and classified into four groups. Thirty-six β-thalassaemia patients treated only with blood transfusion (group I). Thirty-six patients treated with transfusion and iron chelation (group II). Thirty-six patients treated with transfusion, iron chelation and subjected to splenectomy (group III). Group IV involved thirty-six apparently healthy age and sex matched children. CBC plus serum levels of ferritin, IgA, complement C3 and C4 were measured along with detection of CD3+, CD4+, CD8+, CD19+ and CD56+ lymphocyte percentages and absolute counts.
Results: IgA levels were significantly higher in thalassaemia patients compared to controls (p<0.001) plus highly significant increase in IgA levels in splenectomized patients than non-splenectomized (p<0.001). Levels of C3 were significantly decreased in all patients compared with controls (p=0.001) with a highly significant decrease in C3 levels in splenectomized patients than non splenectomized ones (p<0.001) but no statistical difference between their C4 levels. Significant statistical differences were revealed regarding CD3+, CD4+ and CD8+ T lymphocyte percentages within thalassaemia groups when compared to each other’s and to controls. Splenectomized patients had higher significant levels regarding serum ferritin (p=0.02) along with CD3+ (p=0.05), CD4+ (p=0.05) and CD8+ (p=0.037) lymphocyte percentages compared to non-splenectomized. CD19+ lymphocyte percentages were significantly higher while CD56+ lymphocyte percentages were significantly lower in all patients compared with controls (p=0.02 and 0.05).
Conclusion: Immune modulation occurs in thalassaemia patients with regional specific variations and is related to variations in treatment modalities.