title: Homozygous beta-thalassaemia: a review of patients who had splenectomy at the Royal Alexandra Hospital for Children, Sydney.
abstract: Twenty-four patients with homozygous beta-thalassaemia who had been splenectomised and currently on treatment were studied retrospectively. They were divided into two groups. Group A: who had splenectomy prior to commencement of any regular blood transfusion. The mean haemoglobin for this group rose from 5.5 gm/dl pre-splenectomy to 7.7 gm/dl post splenectomy (p < 0.001). Group B: who were on regular blood transfusion when they had their splenectomy and the mean blood transfusion requirement dropped from 317 ml/kg/yr to 230 ml/kg/yr of packed red cells following splenectomy (p < 0.001). Three patients who were on regular blood transfusion and desferrioxamine developed Yersinia enterocolitica infection. They presented with fever and signs of an acute abdomen. At laparotomy, 2 of the patients had acute appendicitis. All 3 appendices grew Yersinia enterocolitica and one patient also had a Yersinia enterocolitica septicaemia. If a patient develops fever and enteritis, desferrioxamine should be stopped temporarily and cotrimoxazole started as prophylaxis against systemic Yersiniosis. No cases of pneumoccocal sepsis was reported.
authors.affiliation: Royal Alexandra Hospital for Children, Sydney, Australia.
authors.forename: T S
authors.initials: TS
authors.lastname: Hoe
authors.forename: A
authors.initials: A
authors.lastname: Lammi
authors.forename: B
authors.initials: B
authors.lastname: Webster
mesh_terms: D000293:Adolescent; D000328:Adult; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006400:Hematocrit; D006454:Hemoglobins / Q000032:analysis; D006720:Homozygote; D006801:Humans; D008297:Male; D013156:Splenectomy; D017086:beta-Thalassemia / Q000097:blood / Q000235:genetics / Q000601:surgery*
chemical_list: D006454:Hemoglobins