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beta-thalassemia (beta-thal) is a worldwide hereditary red cell disorder characterized by severe chronic anemia. Recently, the pyruvate kinase (PK) activator mitapivat has been shown to improve anemia and ineffective erythropoiesis in a mouse model of beta-thal and in non-transfusion dependent thalassemic patients. Here, we showed that in vitro CD34+ derived erythroblasts from beta-thal (codb039) patients are characterized by persistent expression of two PK isoforms, PKR and PKM2, when compared to healthy cells.
View Article and Find Full Text PDFAnn Hematol
March 2025
Departamento de Genética e Evolução - Laboratório de Bioquímica e Genética Aplicada, Universidade Federal de São Carlos (UFSCar), São Carlos, Brazil.
Sickle cell anemia (SCA) presents a complex interplay of factors, with the production of high levels of reactive oxygen species (ROS) and the chronic inflammatory process leading to chronic oxidative stress. In this context, efficient action of antioxidant systems becomes crucial, with particular emphasis on peroxiredoxins (PRDXs) due to their abundance and vital roles. Our primary objective was to establish associations between gene and protein expression of PRDXs 1, 2, and 6, as well as their reducers TRX1, TRXR1, and SRX1, with the characteristic hyperoxidative status observed in SCA patients.
View Article and Find Full Text PDFTurk J Pediatr
February 2025
Section of Hematology, Department of Pediatrics, Faculty of Medicine, Gazi University, Ankara, Türkiye.
Background: In transfusion-dependent forms of β-thalassemia, chronic anemia and iron overload lead to the development of oxidative stress-related DNA damage. In β-thalassemia minor (β-Tm), oxidative stress resulting from an unbalanced globin chain ratio has been documented, even in the absence of anemia and its complications. However, the status of oxidative stress-related DNA damage has not yet been elucidated.
View Article and Find Full Text PDFNutrients
February 2025
Postgraduate Program in Neuroscience and Cell Biology, Federal University of Pará/UFPA, Rua Augusto Corrêa 01, Bairro Guamá, Belém 66075-110, PA, Brazil.
Chronic treatment with dapsone (DDS) has been linked to adverse reactions involving all organ systems, such as dapsone hypersensitivity syndrome, methemoglobinemia and hemolytic anemia, besides neuroinflammation and neurodegeneration due to iron accumulation and oxidative stress. These effects probably occur due to the presence of its toxic metabolite DDS-NOH, which can generate reactive oxygen species (ROS) and iron overload. In this sense, antioxidant compounds with chelating properties, such as alpha-lipoic acid (ALA), may be an interesting adjuvant therapy strategy in treating or preventing these effects.
View Article and Find Full Text PDFHematology
December 2025
Department of Medicine, University of Washington, Seattle, WA, United States.
Introduction: Cases of warm autoimmune hemolytic anemia (wAIHA) often present with life-threatening levels of hemoglobin requiring red blood cell (RBC) transfusion support.
Aim: This literature review assessed the occurrence, safety, effectiveness, and hospitalization burden of RBC transfusions in the management of patients with wAIHA.
Methods: Electronic databases (Embase, MEDLINE) were searched from inception to December 2021 along with additional searches conducted up to March 2024.
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