부산대학교 도서관

OBJECTIVE: To determine the serum transferrin receptor (sTfR) level in patients with Hb-E-β-thalassaemia attending a paediatric haematology clinic. METHODS: Sixty-four patients with Hb-E-β-thalassaemia aged 2.9–10.8 years were attending a paediatric haematology clinic at King Chulalongkorn Memorial Hospital, Bangkok, Thailand. Circulating transferrin receptor was measured, as was the complete blood count and percentage of reticulocytes. A transfusion regimen recommended by the Thalassaemia International Federation was used whenever parents agreed to the treatment. RESULTS: In patients with Hb-E-β-thalassaemia, the concentration of sTfR varied from 1.88 to 28.44 mg/L (mean ± SD 13.99 ± 5.37), while in the control group, it varied from 0.95 to 2.56 mg/L (mean ± SD 1.58 ± 0.41; n = 31). The sTfR level correlated inversely to a single measurement of haemoglobin in the same specimen (r = −0.433; P = 0.00) and was elevated compared to the control group (P = 0.00; α < 0.05). In addition, the sTfR level correlated inversely to mean pre-transfusion haemoglobin (r = −0.36; P = 0.003). CONCLUSIONS: The level of sTfR is elevated in children with Hb-E-β-thalassaemia. The sTfR level can be used as an adjunct real-time assessment of erythropoietic activity, comparable with mean pre-transfusion haemoglobin. Measurement of sTfR is easy using most automated chemical analyzers, and material can be obtained from routine specimen collections. Further studies should be carried out in a larger population to verify the benefits of this test to patients.