title: 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 A:5 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.
authors.affiliation: Division of Pediatric Hematology, Pramongkutklao College of Medicine, Bangkok, Thailand.
authors.forename: K
authors.initials: K
authors.lastname: Torcharus
authors.forename: W
authors.initials: W
authors.lastname: Withayathawornwong
authors.forename: T
authors.initials: T
authors.lastname: Sriphaisal
authors.forename: T
authors.initials: T
authors.lastname: Krutvacho
authors.forename: P
authors.initials: P
authors.lastname: Arnutti
authors.forename: C
authors.initials: C
authors.lastname: Suwanasophorn
mesh_terms: D000293:Adolescent; D001803:Blood Transfusion / Q000379:methods; D002648:Child; D002675:Child, Preschool; D003676:Deferoxamine / Q000627:therapeutic use; D005260:Female; D005293:Ferritins / Q000097:blood; D006446:Hemoglobin E; D006453:Hemoglobinopathies / Q000097:blood / Q000150:complications / Q000628:therapy*; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D008297:Male; D013156:Splenectomy; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications / Q000628:therapy*
chemical_list: D006454:Hemoglobins; D005293:Ferritins; D006446:Hemoglobin E; D003676:Deferoxamine