Khairullah, Shasha and Jackson, N. (2021) Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia. Medical Journal of Malaysia, 76 (1). pp. 41-45. ISSN 03005283,
Full text not available from this repository.Abstract
Non-transfused β-thalassaemia patients develop complications related to unsuppressed ineffective erythropoiesis (IE). Serum markers of IE would be useful for risk stratification and monitoring treatment. We studied β-thalassaemia trait (β-TT) and non-transfusion-dependent β-thalassaemia (β-NTDT) patients. Serum erythropoietin (EPO) and soluble transferrin receptor (sTfR) were correlated against markers of clinical severity (haemoglobin, LDH, retics, bilirubin, spleen size) and iron overload (ferritin, hepcidin, and MRI-T2* in NTDT patients). Eleven β-NTDT and nine β-TT subjects were studied. β-NTDT patients had significantly higher markers of haemolysis and iron overload. In β-NTDT, liver iron ranged from mild to severe, but no cardiac loading was seen. EPO and sTfR were higher in patients with β-NTDT than β-TT, and correlated significantly with each other (ρ=0.630, p=0.003). Both markers were negatively correlated with haemoglobin (sTfR ρ=-0.540, p=0.014; EPO ρ=-0.807, p<0.001, and positively correlated with spleen size (sTfR ρ=0.783, p<0.001; EPO ρ=0.654, p=0.002) and markers of iron overload. There was a strong correlation between ferritin and hepcidin (ρ=0.720, p<0.001), and a relatively lower increment of hepcidin for the degree of iron overload in β-NTDT compared to β-TT. EPO and sTfR appear to be reliable markers of erythropoiesis in non-transfused β-thalassaemia and correlate well with markers of disease severity. Their role in managing patients, predicting complications, and monitoring response to treatments aimed at reducing IE should be explored. © 2021, Malaysian Medical Association. All rights reserved.
| Item Type: | Article |
|---|---|
| Funders: | UNSPECIFIED |
| Uncontrolled Keywords: | Ineffective erythropoiesis; Non-transfusion-dependent thalassaemia; β-thalassaemia intermedia |
| Subjects: | R Medicine > R Medicine (General) R Medicine > RA Public aspects of medicine |
| Divisions: | Faculty of Medicine Faculty of Medicine > Medicine Department |
| Depositing User: | Ms Zaharah Ramly |
| Date Deposited: | 27 Nov 2023 07:07 |
| Last Modified: | 29 Nov 2023 03:53 |
| URI: | http://eprints.um.edu.my/id/eprint/35815 |
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