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Thalassemia Reports
Special Issues
Thalassemia Syndromes in Developing Countries: Has Anything Changed?
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Thalassemia Syndromes in Developing Countries: Has Anything Changed?

A special issue of Thalassemia Reports (ISSN 2039-4365).

Deadline for manuscript submissions: closed (31 December 2023) | Viewed by 12380

Special Issue Editor

Dr. Anuja P. Premawardhena

E-Mail Website
Guest Editor
Faculty of Medicine, University of Kelaniya, Kelaniya, Sri Lanka
Interests: thalassemia

Special Issue Information

Dear Colleagues,

With improving blood transfusion services and provision of adequate chelation in well-supervised settings, the outlook of survival of patients with thalassaemia has significantly improved in Mediterranean countries and other resource-rich settings. Newer medications to raise haemoglobin are being trialled and now gradually used in these centres with some efficacy. But have these positive trends transcended to those cities and countries that have the bulk of thalassaemia patients in the world? Almost half a century after the widespread use of desferrioxamine began, has it reached to the patients who most need it? MRI is the most widely used technique for organ-based iron measurements—how widely used are such tools in the most vulnerable of populations? Has the quality of life in patients with thalassaemia syndromes improved in the developing world?

Dr. Anuja P. Premawardhena
Guest Editor

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Keywords

  • haemoglobinopathies
  • thalassaemia
  • survival
  • quality of life
  • developing world

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Published Papers (3 papers)

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Research

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13 pages, 1385 KiB  
Article
Spectrum of Thalassemia and Hemoglobinopathy Using Capillary Zone Electrophoresis: A Facility-Based Single Centred Study at icddr,b in Bangladesh
by Anamul Hasan, Jigishu Ahmed, Bikash Chandra Chanda, Maisha Aniqua, Raisa Akther, Palash Kanti Dhar, Kazi Afrin Binta Hasan, Abdur Rouf Siddique, Md. Zahidul Islam, Sharmine Zaman Urmee and Dinesh Mondal
Thalass. Rep. 2023, 13(2), 131-143; https://doi.org/10.3390/thalassrep13020012 - 10 May 2023
Viewed by 4780
Abstract
Background: Although the global thalassemia zone covers Bangladesh, there are very limited studies conducted in this region. Therefore, the focus of our study is to understand the prevalence and burden of thalassemia and hemoglobinopathy in Bangladesh. Methods: The analysis was based on a [...] Read more.
Background: Although the global thalassemia zone covers Bangladesh, there are very limited studies conducted in this region. Therefore, the focus of our study is to understand the prevalence and burden of thalassemia and hemoglobinopathy in Bangladesh. Methods: The analysis was based on a retrospective evaluation of laboratory diagnoses between 2007 January and 2021 October. A total of 8503 specimens were sampled and analyzed which were either referred by corresponding physicians or self-referred. This was neither any epidemiological nationwide survey nor was the study population chosen randomly. Hematological data were obtained through capillary zone electrophoresis and corresponding complete blood count. Results: 1971 samples (~23.18% of the total) were found with at least one inherited hemoglobin disorder. The most common hemoglobin disorder observed was the hemoglobin E (Hb E) trait (10.67%), followed by the β-thalassemia trait (8.4%), homozygotic Hb E (1.59%), and Hb E/β-thalassemia (1.58%). Other variants found in this study with minimal percentages were Hb N-Seattle, Hb S, Hb D-Punjab, Hb Lepore, Hb C, Hb Hope, Hb H, and hereditary persistence of fetal hemoglobin. Discussion: The pattern of thalassemia and hemoglobinopathy in our study is diverse and heterogeneous. A broad and detailed spectrum of such inherited hemoglobin disorders will ultimately be helpful in implementing nationwide thalassemia management and strategy policy in Bangladesh. Full article
(This article belongs to the Special Issue Thalassemia Syndromes in Developing Countries: Has Anything Changed?)
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Review

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16 pages, 1005 KiB  
Review
Understanding the Intricacies of Iron Overload Associated with β-Thalassemia: A Comprehensive Review
by Subhangi Basu, Motiur Rahaman, Tuphan Kanti Dolai, Praphulla Chandra Shukla and Nishant Chakravorty
Thalass. Rep. 2023, 13(3), 179-194; https://doi.org/10.3390/thalassrep13030017 - 3 Jul 2023
Cited by 4 | Viewed by 4067
Abstract
β-thalassemia, a congenital genetic hematological disorder characterized by the decrease or absence of β-globin chains, leads to a decrease in levels of Hemoglobin A. The affected individuals can be categorized into two cohorts based on transfusion dependency: transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia [...] Read more.
β-thalassemia, a congenital genetic hematological disorder characterized by the decrease or absence of β-globin chains, leads to a decrease in levels of Hemoglobin A. The affected individuals can be categorized into two cohorts based on transfusion dependency: transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT). Remarkably, despite the primary pathology lying in β-globin chain depletion, β-thalassemia also exhibits an intriguing association with iron overload. Iron metabolism, a tightly regulated physiological process, reveals a complex interplay in these patients. Over time, both cohorts of β-thalassemic individuals develop iron overload, albeit through distinct mechanisms. Addressing the diverse complications that arise due to iron overload in β-thalassemic patients, the utilization of iron chelators has gained a lot of significance. With varying efficacies, routes of administration, and modes of action, different iron chelators offer unique benefits to patients. In the Indian context, three commercialized iron chelators have emerged, showcasing a high adherence rate to iron chelator-based treatment regimens among β-thalassemic individuals. In this review, we explore the intriguing connection between β-thalassemia and iron overload, shedding light on the intricate mechanisms at play. We delve into the intricacies of iron metabolism, unveiling the distinct pathways leading to iron accumulation in these patients. Additionally, the therapeutic efficacy of different iron chelators in managing iron overload complications is mentioned briefly, along with the guidelines for their usage in India. Through this comprehensive analysis, we aim to deepen our understanding of β-thalassemia and iron overload, paving the way for optimized treatment strategies. Ultimately, our findings provide valuable insights into improving the care and outcomes of individuals affected by β-thalassemia. Full article
(This article belongs to the Special Issue Thalassemia Syndromes in Developing Countries: Has Anything Changed?)
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Other

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8 pages, 707 KiB  
Perspective
What Is the Relevance of Murburn Concept in Thalassemia and Respiratory Diseases?
by Kelath Murali Manoj
Thalass. Rep. 2023, 13(2), 144-151; https://doi.org/10.3390/thalassrep13020013 - 12 May 2023
Viewed by 2636
Abstract
Murburn concept is a novel perspective for understanding cellular function, deeming cells as simple chemical engines (SCE) that are powered by redox reactions initiated by effective charge separation (ECS). The 1-electron active diffusible reactive (oxygen) species, or DR(O)S, equilibriums involved in these processes [...] Read more.
Murburn concept is a novel perspective for understanding cellular function, deeming cells as simple chemical engines (SCE) that are powered by redox reactions initiated by effective charge separation (ECS). The 1-electron active diffusible reactive (oxygen) species, or DR(O)S, equilibriums involved in these processes are also crucial for homeostasis, coherently networking cells, and rendering electromechanical functions of sensing and responding to stimuli. This perspective presents the true physiological function of oxygen, which is to enable ECS and the generation of DR(O)S. Therefore, DR(O)S must now to be seen as the quintessential elixir of life, although they might have undesired effects (i.e., the traditionally perceived oxidative stress) when present in the wrong amounts, places and times. We also elaborated that tetrameric hemoglobin (Hb) is actually an ATP-synthesizing murzyme (an enzyme working via murburn concept) and postulated that several post-translational modifications (such as glycation) on Hb could result from murburn activity. Murburn perspective has also enabled the establishment of a facile rationale explaining the sustenance of erythrocytes for 3–4 months, despite their lacking nucleus or mitochondria (to coordinate their various functions and mass-produce ATP, respectively). Although thalassemia has its roots in genetic causation, the new awareness of the mechanistic roles of oxygen-hemoglobin-erythrocyte trio significantly impacts our approaches to interpreting research data and devising therapies for this malady. These insights are also relevant in other clinical manifestations that involve respiratory distress (such as asthma, lung cancer, COVID-19 and pneumonia) and mitochondrial diseases. Herein, these contexts and developments are briefly discussed. Full article
(This article belongs to the Special Issue Thalassemia Syndromes in Developing Countries: Has Anything Changed?)
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