Acute hemolytic transfusion reaction mediated by anti-Jk<sup>b</sup> antibody in a pediatric patient with β-thalassemia major: a case report illustrating an anamnestic alloimmune response. [PDF]
Xu J, Chen J.
europepmc +1 more source
Persistent thrombocytosis in β-thalassemia post-splenectomy: A STROBE-compliant retrospective cohort study at a Jordanian referral center. [PDF]
Alesufi A +10 more
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Low- to moderate-dose thalidomide combination rescue after luspatercept failure in transfusion-dependent β-thalassemia: a real-world cohort study. [PDF]
Nanthachak S +5 more
europepmc +1 more source
β-Thalassemia is caused by reduced (β +) or absent (β 0) synthesis of the β-globin chains of hemoglobin. Three clinical and hematological conditions of increasing severity are recognized: the β-thalassemia carrier state, thalassemia intermedia, and ...
Raffaella Origa
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Global distribution of β-thalassemia mutations: An Update.
Gene, 2023One excellent illustration of how a single gene abnormality may result in a spectrum of disease incidence is the incredible phenotypic variety of β-thalassemia, which spans from severe anemia and transfusion needs to an utterly asymptomatic sickness ...
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Pretransfusion hemoglobin level and mortality in adults with transfusion-dependent β-thalassemia.
Blood, 2023In a large cohort of patients with transfusion-dependent β-thalassemia, we demonstrate an association between ascending pretransfusion hemoglobin levels starting at 9.5 g/dL and reduced mortality risk.
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Prevalence and Management of β-Thalassemia in India
Hemoglobin, 2022India bears a huge burden of hemoglobinopathies, and the most prevalent is thalassemia. The different types of thalassemia include minor, major and intermedia, based on the α/β-globin chain inequality.
S. Yadav, P. Panchal, Kavitha Menon
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Thalassemia is a wide range hereditary disease with high incidence in Egypt along with the high frequency of consanguineous marriages. Investigation the heterogeneity, molecular basis, and natural history are the most effective methods to deal with the thalassemia to develop effective method for management and prevention including the prenatal ...
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A Phase 3 Trial of Luspatercept in Patients with Transfusion-Dependent β-Thalassemia.
New England Journal of Medicine, 2020BACKGROUND Patients with transfusion-dependent β-thalassemia need regular red-cell transfusions. Luspatercept, a recombinant fusion protein that binds to select transforming growth factor β superfamily ligands, may enhance erythroid maturation and reduce
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Exagamglogene Autotemcel for Transfusion-Dependent β-Thalassemia.
New England Journal of MedicineBACKGROUND Exagamglogene autotemcel (exa-cel) is a nonviral cell therapy designed to reactivate fetal hemoglobin synthesis through ex vivo clustered regularly interspaced short palindromic repeats (CRISPR)-Cas9 gene editing of the erythroid-specific ...
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