High transfusion in children with beta-thalassemia/Hb E: clinical and laboratory assessment of 18 cases.

ABSTRACT

Between 1990-1992, 18 patients with beta-thalassemia/Hb E age between 2-13 years (mean 7.4 +/- 3 years) were examined. Three patients were splenectomized and the rest were nonsplenectomized. They were divided into 3 groups. Group A5 nonsplenectomized and 3 splenectomized patients had high transfusion rates with subcutaneous desferrioxamine injections. Five patients in group B received only high transfusion whereas in 5 patients in group C the levels pretransfusion Hb were maintained between 6-7 g/dl. The mean blood consumption in the nonsplenectomized groups were 220 +/- 25.3, 221 +/- 59 and 175.4 +/- 45.4 ml/kg/year in groups A, B and C, respectively. In group A, the mean blood requirement was 40% higher in the nonsplenectomized group. In the high transfusion regimen the spleen size did not increase and serum aspartate aminotransferase showed a striking fall in the majority of cases. The absolute increases in serum ferritin were 843.2 +/- 395, 861 +/- 252 and 1,262 +/- 440 ng/ml in groups A, B and C, respectively. These data demonstrated that high transfusion with desferrioxamine injection could improve the clinical well being of the patients.