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Thalassemia Reports
Volume 14
Issue 3
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Thalass. Rep., Volume 14, Issue 3 (September 2024) – 3 articles

Cover Story (view full-size image): β-thalassemia, a life-threatening inheritable hemoglobin disorder caused by mutations in the HBB gene, poses a significant public health challenge in the world. Although no comprehensive work has been carried out in Bangladesh, the world prevalence and small-scale works have indicated the possibility of a high prevalence of this disease in the country. Therefore, this review aims to explore the present situation of β-thalassemia in Bangladesh and propose approaches to mitigate its impact in the future. Limited awareness, a high incidence of consanguineous marriage, and inadequate access to healthcare are the possible factors responsible for the high prevalence of thalassemia in Bangladesh, while the absence of public health policy and a national health insurance system further exacerbate the situation. View this paper
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10 pages, 1201 KiB  
Article
The Effect of Resveratrol on Gamma Globin Gene Expression in Patients with Beta Thalassemia: The Role of Adaptation to Cellular Stress
by Hossein Jalali, Mohammad Reza Mahdavi, Mehrnoush Kosaryan, Ahmad Najafi, Aily Aliasgharian and Ebrahim Salehifar
Thalass. Rep. 2024, 14(3), 71-80; https://doi.org/10.3390/thalassrep14030009 - 17 Sep 2024
Viewed by 473
Abstract
HbF induction is an appropriate strategy to ameliorate the severity of β-thalassemia symptoms. Hydroxyurea (HU) is the most common chemical agent introduced as an HbF inducer but responsiveness to HU is variable and the introduction of HbF inducers alternative to HU with low [...] Read more.
HbF induction is an appropriate strategy to ameliorate the severity of β-thalassemia symptoms. Hydroxyurea (HU) is the most common chemical agent introduced as an HbF inducer but responsiveness to HU is variable and the introduction of HbF inducers alternative to HU with low cytotoxicity has been a crucial challenge. Resveratrol is an HbF inducer agent that may have favorable effects on the differentiation of hematopoietic erythroid progenitors (HEPs). The present study aimed to investigate the effect of resveratrol on γ-globin, stress response, and anti-apoptotic gene expression among hydroxyurea (HU)-responders and HU-nonresponders (HU-NR). Four cases of HU-R and four cases of HU-NR were studied. HEPs of the patients were cultured, and the expression of γ-globin, Foxo3, and Bclxl was assessed. Moreover, the differentiation and apoptotic rate of the cells were investigated using flow cytometry analysis. In three cases, the γ-globin gene expression increased after resveratrol treatment. All of the HU-NR patients were also non-responders to resveratrol (Res-NR). The expression of Foxo3 and Bclxl genes was higher in responders to resveratrol (Res-R) compared to non-responders (Res-NR). The rate of apoptosis in Res-R patients was also lower than in Res-NR. Responders to resveratrol also had a higher rate of HEP maturation. The cells of both HU–NR and Res-NR patients could not adapt to stress conditions and proceed to the erythroid differentiation. In conclusion, resveratrol increased the γ-globin expression in HEPs of β-thalassemia patients. The response was observed only in R-HU patients with similar cellular pathways. Full article
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11 pages, 248 KiB  
Article
Sociodemographic Determinants of Adherence and Treatment Efficacy in Paediatric Thalassemia Patients from Sarbaz-Rask, Iran
by Atousa Babamohammadi, QiYuee Wang, Elham Mohajeri and Saeid Esmaeilian
Thalass. Rep. 2024, 14(3), 60-70; https://doi.org/10.3390/thalassrep14030008 - 15 Aug 2024
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Abstract
Background: The effective management of iron overload in transfusion-dependent thalassemia (TDT) requires adherence to iron chelation therapy (ICT). However, adherence rates among pediatric thalassemia patients remain suboptimal. This study aimed to evaluate adherence levels and identify sociodemographic and clinical factors impacting ICT adherence [...] Read more.
Background: The effective management of iron overload in transfusion-dependent thalassemia (TDT) requires adherence to iron chelation therapy (ICT). However, adherence rates among pediatric thalassemia patients remain suboptimal. This study aimed to evaluate adherence levels and identify sociodemographic and clinical factors impacting ICT adherence in pediatric TDT patients from Sarbaz-Rask, Iran. Methods: This cross-sectional study assessed 58 pediatric TDT patients aged 2–18 years at a thalassemia clinic from April 2021 to March 2022. Adherence was evaluated using the medication possession ratio. Logistic regression and correlation analyses identified predictors of adherence and treatment efficacy based on serum ferritin levels. Results: Adherence was satisfactory in 58.6% of patients and associated with younger maternal age (93.8% for 18–30 years, p = 0.008) and urban residency (p = 0.02). Logistic regression identified urban residency (OR = 20.265, p = 0.073) and a maternal age of 18–30 years (OR = 39.236, p = 0.005) as key predictors of adherence. Adherence was not significantly influenced by having a sibling with thalassemia or the maternal educational level. Treatment efficacy was observed in 27.6% of patients. Maternal age impacted adherence in poorly controlled patients (p = 0.007). Urban residents showed higher adherence rates, particularly with poor control (p = 0.017). Conclusions: Younger maternal age and urban residency emerged as positive predictors of adherence and treatment efficacy in pediatric thalassemia patients from Sarbaz-Rask. Targeted interventions supporting rural families and those with older maternal caregivers may improve adherence and outcomes in this population. Full article
11 pages, 274 KiB  
Review
β-Thalassemia in Bangladesh: Current Status and Future Perspectives
by Arnob Mitro, Didar Hossain, Md Muhibur Rahman, Beauty Dam and Mohammad Jakir Hosen
Thalass. Rep. 2024, 14(3), 49-59; https://doi.org/10.3390/thalassrep14030007 - 8 Jul 2024
Viewed by 1861
Abstract
β-thalassemia, a life-threatening inheritable hemoglobin disorder caused by mutations in the HBB gene, poses a significant public health challenge in the world. Although no comprehensive work has been carried out in Bangladesh, the world prevalence and small-scale works indicated the possibility of a [...] Read more.
β-thalassemia, a life-threatening inheritable hemoglobin disorder caused by mutations in the HBB gene, poses a significant public health challenge in the world. Although no comprehensive work has been carried out in Bangladesh, the world prevalence and small-scale works indicated the possibility of a high prevalence of this disease in the country. Therefore, this review aims to explore the present situation of β-thalassemia in Bangladesh and propose approaches to mitigate its impact in the future. Limited awareness, a high incidence of consanguineous marriage, and inadequate access to healthcare are possible factors responsible for the high prevalence of thalassemia in Bangladesh, while the absence of public health policy and a national health insurance system further exacerbate the situation. The understanding of the genetic landscape and modern treatment strategies for β-thalassemia is hindered by the lack of comprehensive data on the mutation spectrum. In addition to conventional therapy such as blood transfusion, advanced practices such as splenectomy, hematopoietic stem cell transplantation, and emerging therapies such as gene therapy show promise for future cures but have yet to be widely implemented in this country. To effectively address the challenges of β-thalassemia, it is crucial to adopt comprehensive strategies, including a public awareness campaign, public health intervention, mandatory premarital screening, genetic counselling, and a national thalassemia prevention program. Additionally, understanding the spectrum of mutations and new therapeutic interventions is crucial for advanced healthcare strategies. Full article
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