Boonchalermvichian C, Paritpokee N, Bhokaisawan N, Nuchprayoon I, Wiwanitkit V
Department of Laboratory Medicine, King Chulalongkorn Memorial Hospital, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
J Paediatr Child Health. 2002 Dec;38(6):601-3. doi: 10.1046/j.1440-1754.2002.00062.x.
To determine the serum transferrin receptor (sTfR) level in patients with Hb-E-beta-thalassaemia attending a paediatric haematology clinic.
Sixty-four patients with Hb-E-beta-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.
In patients with Hb-E-beta-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; alpha < 0.05). In addition, the sTfR level correlated inversely to mean pre-transfusion haemoglobin (r = - 0.36; P = 0.003).
The level of sTfR is elevated in children with Hb-E-beta-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.
测定在儿科血液科诊所就诊的血红蛋白E-β地中海贫血患者的血清转铁蛋白受体（sTfR）水平。
64例年龄在2.9至10.8岁的血红蛋白E-β地中海贫血患者在泰国曼谷朱拉隆功国王纪念医院的儿科血液科诊所就诊。检测循环转铁蛋白受体，同时检测全血细胞计数和网织红细胞百分比。只要家长同意治疗，就采用地中海贫血国际联合会推荐的输血方案。
血红蛋白E-β地中海贫血患者的sTfR浓度在1.88至28.44mg/L之间（平均值±标准差为13.99±5.37），而对照组的浓度在0.95至2.56mg/L之间（平均值±标准差为1.58±0.41；n = 31）。sTfR水平与同一样本中单次血红蛋白测量值呈负相关（r = - 0.433；P = 0.00），且与对照组相比升高（P = 0.00；α < 0.05）。此外，sTfR水平与输血前平均血红蛋白呈负相关（r = - 0.36；P = 0.003）。
血红蛋白E-β地中海贫血患儿的sTfR水平升高。sTfR水平可作为红细胞生成活性的辅助实时评估指标，与输血前平均血红蛋白相当。使用大多数自动化学分析仪检测sTfR很容易，且材料可从常规标本采集中获得。应在更大规模人群中进行进一步研究，以验证该检测对患者的益处。
