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Marked increase in serum transferrin receptor among Thai children with Hb-E-beta-thalassaemia
C Boonchalermvichian1, N Paritpokee, N Bhokaisawan
1Department of Laboratory Medicine, King Chulalongkorn Memorial Hospital, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand. chaiyaporn_b@wmi.usyd.edu.au
Insights
Serum transferrin receptor (sTfR) levels are elevated in children with Hb-E-beta-thalassaemia, indicating increased erythropoietic activity. This finding suggests sTfR can serve as a valuable tool for assessing red blood cell production in these patients.
Area of Science:
- Paediatric Haematology
- Molecular Biology
- Biochemistry
Background:
- Beta-thalassaemia is a significant public health concern, particularly in regions with a high prevalence of hemoglobinopathies.
- Hb-E-beta-thalassaemia, a common variant, presents with a range of clinical severity requiring careful monitoring of erythropoietic activity.
- Serum transferrin receptor (sTfR) is a marker of cellular iron uptake and erythropoiesis.
Purpose of the Study:
- To determine the serum transferrin receptor (sTfR) levels in children diagnosed with Hb-E-beta-thalassaemia.
- To evaluate the correlation between sTfR levels and haemoglobin concentration in these patients.
- To assess the utility of sTfR as an indicator of erythropoietic activity in Hb-E-beta-thalassaemia.
Main Methods:
- A cohort of 64 children (2.9-10.8 years) with Hb-E-beta-thalassaemia attending a paediatric haematology clinic were enrolled.
- Serum transferrin receptor (sTfR) levels were measured along with complete blood counts and reticulocyte percentages.
- Data were compared with a control group (n=31), and correlations with haemoglobin and pre-transfusion haemoglobin were analyzed.
Main Results:
- Patients with Hb-E-beta-thalassaemia exhibited significantly elevated sTfR levels (mean 13.99 +/- 5.37 mg/L) compared to controls (mean 1.58 +/- 0.41 mg/L).
- sTfR levels showed a significant inverse correlation with haemoglobin concentration (r = -0.433, P = 0.00) and mean pre-transfusion haemoglobin (r = -0.36, P = 0.003).
- The elevated sTfR levels were statistically significant compared to the control group (P = 0.00).
Conclusions:
- Serum transferrin receptor (sTfR) levels are markedly elevated in children with Hb-E-beta-thalassaemia.
- sTfR serves as a reliable adjunct marker for assessing erythropoietic activity, comparable to mean pre-transfusion haemoglobin.
- The ease of measurement using automated analyzers and routine specimen collection makes sTfR a practical diagnostic tool.
Objective:
To determine the serum transferrin receptor (sTfR) level in patients with Hb-E-beta-thalassaemia attending a paediatric haematology clinic.
Methods:
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.
Results:
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).
Conclusions:
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.
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