Impaired Terminal Erythroid Maturation in β<sup>0</sup>-Thalassemia/HbE Patients with Different Clinical Severity
Issued Date
2022-04-01
Resource Type
eISSN
20770383
Scopus ID
2-s2.0-85126932498
Journal Title
Journal of Clinical Medicine
Volume
11
Issue
7
Rights Holder(s)
SCOPUS
Bibliographic Citation
Journal of Clinical Medicine Vol.11 No.7 (2022)
Suggested Citation
Suriyun T., Winichagoon P., Fucharoen S., Sripichai O. Impaired Terminal Erythroid Maturation in β<sup>0</sup>-Thalassemia/HbE Patients with Different Clinical Severity. Journal of Clinical Medicine Vol.11 No.7 (2022). doi:10.3390/jcm11071755 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/87340
Title
Impaired Terminal Erythroid Maturation in β<sup>0</sup>-Thalassemia/HbE Patients with Different Clinical Severity
Author(s)
Other Contributor(s)
Abstract
Anemia in β-thalassemia is associated with ineffective erythropoiesis and a shortened lifespan of erythroid cells. The limited differentiation of β-thalassemic erythroblasts has been documented, but the characteristic feature of terminal erythroid maturation and its physiological relevance are not clearly described in β-thalassemias. Here, the red blood cell and reticulocyte cellular characteristics were determined in patients with β0-thalassemia/HbE in comparison to patients with iron deficiency anemia and healthy normal subjects. Severely affected β0-thalassemia/HbE patients showed the highest increase in immature reticulocytes, but the number of total erythrocytes was the lowest. Despite similar ranges of hemoglobin levels, β0-thalassemia/HbE patients had a higher number of reticulocytes and a greater proportion of immature fraction than patients with iron deficiency anemia did. In vitro CD34+ hematopoietic progenitor cells’ culture and flow cytometry analysis were conducted to investigate the erythroid maturation and mitochondrial clearance in β0-thalassemia/HbE erythroid cells as compared to normal cells. The delayed erythroid maturation and evidence of impaired mitochondria clearance were observed in β0-thalassemia/HbE cells at the terminal stage of differentiation. Additionally, increased transcript levels of genes related to erythroid mitophagy, BNIP3L and PINK1, were revealed in β0-thalassemia/HbE erythroblasts. The findings indicate that the erythroid maturation is physiologically relevant, and that the restoration of terminal maturation represents a potential therapeutic target for β-thalassemias.