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Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia

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) pat...

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Main Authors:	Khairullah, Shasha, Jackson, N.
Format:	Article
Published:	Malaysian Medical Association 2021
Subjects:	R Medicine (General) RA Public aspects of medicine
Online Access:	http://eprints.um.edu.my/35815/ https://www.scopus.com/inward/record.uri?eid=2-s2.0-85100093625&partnerID=40&md5=7a4f4c9793ab5a0c6d556d125610d024
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spelling	my.um.eprints.358152023-11-29T03:53:16Z http://eprints.um.edu.my/35815/ Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia Khairullah, Shasha Jackson, N. R Medicine (General) RA Public aspects of medicine 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. Malaysian Medical Association 2021 Article PeerReviewed 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, https://www.scopus.com/inward/record.uri?eid=2-s2.0-85100093625&partnerID=40&md5=7a4f4c9793ab5a0c6d556d125610d024
institution	Universiti Malaya
building	UM Library
collection	Institutional Repository
continent	Asia
country	Malaysia
content_provider	Universiti Malaya
content_source	UM Research Repository
url_provider	http://eprints.um.edu.my/
topic	R Medicine (General) RA Public aspects of medicine
spellingShingle	R Medicine (General) RA Public aspects of medicine Khairullah, Shasha Jackson, N. Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia
description	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.
format	Article
author	Khairullah, Shasha Jackson, N.
author_facet	Khairullah, Shasha Jackson, N.
author_sort	Khairullah, Shasha
title	Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia
title_short	Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia
title_full	Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia
title_fullStr	Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia
title_full_unstemmed	Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia
title_sort	markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia
publisher	Malaysian Medical Association
publishDate	2021
url	http://eprints.um.edu.my/35815/ https://www.scopus.com/inward/record.uri?eid=2-s2.0-85100093625&partnerID=40&md5=7a4f4c9793ab5a0c6d556d125610d024
_version_	1783876640358531072
score	13.211869

Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia

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