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  1. Article ; Online: Diffuse myocardial fibrosis as an SCD biomarker.

    Thein, Swee Lay / Sachdev, Vandana

    Blood

    2022  Volume 140, Issue 11, Page(s) 1191–1192

    MeSH term(s) Anemia, Sickle Cell/pathology ; Biomarkers ; Cardiomyopathies/pathology ; Fibrosis ; Humans ; Myocardium/pathology
    Chemical Substances Biomarkers
    Language English
    Publishing date 2022-09-15
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2022017725
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Revisiting anemia in sickle cell disease and finding the balance with therapeutic approaches.

    Xu, Julia Zhe / Thein, Swee Lay

    Blood

    2022  Volume 139, Issue 20, Page(s) 3030–3039

    Abstract: Chronic hemolytic anemia and intermittent acute pain episodes are the 2 hallmark characteristics of sickle cell disease (SCD). Anemia in SCD not only signals a reduction of red cell mass and oxygen delivery, but also ongoing red cell breakdown and ... ...

    Abstract Chronic hemolytic anemia and intermittent acute pain episodes are the 2 hallmark characteristics of sickle cell disease (SCD). Anemia in SCD not only signals a reduction of red cell mass and oxygen delivery, but also ongoing red cell breakdown and release of cell-free hemoglobin, which together contribute to a number of pathophysiological responses and play a key role in the pathogenesis of cumulative multiorgan damage. However, although anemia is clearly associated with many detrimental outcomes, it may also have an advantage in SCD in lowering risks of potential viscosity-related complications. Until recently, clinical drug development for SCD has predominantly targeted a reduction in the frequency of vaso-occlusive crises as an endpoint, but increasingly, more attention is being directed toward addressing the contribution of chronic anemia to poor outcomes in SCD. This article aims to explore the complex pathophysiology and mechanisms of anemia in SCD, as well as the need to balance the benefits of raising hemoglobin levels with the potential risks of increasing blood viscosity, in the context of the current therapeutic landscape for anemia in SCD.
    MeSH term(s) Acute Pain ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/therapy ; Blood Viscosity ; Erythrocytes/metabolism ; Humans
    Language English
    Publishing date 2022-05-19
    Publishing country United States
    Document type Journal Article
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2021013873
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  3. Article ; Online: Targeting ZNF410 as a potential β-hemoglobinopathy therapy.

    Tumburu, Laxminath / Thein, Swee Lay

    Nature genetics

    2021  Volume 53, Issue 5, Page(s) 589–590

    MeSH term(s) Fetus ; Globins ; Hemoglobinopathies/genetics ; Hemoglobinopathies/therapy ; Humans
    Chemical Substances Globins (9004-22-2)
    Language English
    Publishing date 2021-04-15
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 1108734-1
    ISSN 1546-1718 ; 1061-4036
    ISSN (online) 1546-1718
    ISSN 1061-4036
    DOI 10.1038/s41588-021-00817-y
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  4. Article ; Online: Recent developments in the use of pyruvate kinase activators as a new approach for treating sickle cell disease.

    Parekh, Dina S / Eaton, William A / Thein, Swee Lay

    Blood

    2023  Volume 143, Issue 10, Page(s) 866–871

    Abstract: Abstract: Pyruvate kinase (PK) is a key enzyme in glycolysis, the sole source of adenosine triphosphate, which is essential for all energy-dependent activities of red blood cells. Activating PK shows great potential for treating a broad range of ... ...

    Abstract Abstract: Pyruvate kinase (PK) is a key enzyme in glycolysis, the sole source of adenosine triphosphate, which is essential for all energy-dependent activities of red blood cells. Activating PK shows great potential for treating a broad range of hemolytic anemias beyond PK deficiency, because they also enhance activity of wild-type PK. Motivated by observations of sickle-cell complications in sickle-trait individuals with concomitant PK deficiency, activating endogenous PK offers a novel and promising approach for treating patients with sickle-cell disease.
    MeSH term(s) Humans ; Pyruvate Kinase/deficiency ; Anemia, Hemolytic, Congenital Nonspherocytic/drug therapy ; Anemia, Hemolytic, Congenital Nonspherocytic/etiology ; Erythrocytes ; Anemia, Sickle Cell/drug therapy ; Anemia, Sickle Cell/complications ; Pyruvate Metabolism, Inborn Errors
    Chemical Substances Pyruvate Kinase (EC 2.7.1.40)
    Language English
    Publishing date 2023-11-14
    Publishing country United States
    Document type Journal Article
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2023021167
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  5. Article ; Online: COVID-19 and sickle cell disease.

    Menapace, Laurel A / Thein, Swee Lay

    Haematologica

    2020  Volume 105, Issue 11, Page(s) 2501–2504

    MeSH term(s) Anemia, Sickle Cell/epidemiology ; Anemia, Sickle Cell/therapy ; Anemia, Sickle Cell/virology ; Betacoronavirus/isolation & purification ; COVID-19 ; Coronavirus Infections/complications ; Coronavirus Infections/transmission ; Coronavirus Infections/virology ; Disease Management ; Humans ; Immunocompromised Host ; Pandemics ; Pneumonia, Viral/complications ; Pneumonia, Viral/transmission ; Pneumonia, Viral/virology ; SARS-CoV-2
    Keywords covid19
    Language English
    Publishing date 2020-11-01
    Publishing country Italy
    Document type Journal Article
    ZDB-ID 2333-4
    ISSN 1592-8721 ; 0017-6567 ; 0390-6078
    ISSN (online) 1592-8721
    ISSN 0017-6567 ; 0390-6078
    DOI 10.3324/haematol.2020.255398
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  6. Article: Genetic Basis and Genetic Modifiers of β-Thalassemia and Sickle Cell Disease.

    Thein, Swee Lay

    Advances in experimental medicine and biology

    2017  Volume 1013, Page(s) 27–57

    Abstract: β-thalassemia and sickle cell disease (SCD) are prototypical Mendelian single gene disorders, both caused by mutations affecting the adult β-globin gene. Despite the apparent genetic simplicity, both disorders display a remarkable spectrum of phenotypic ... ...

    Abstract β-thalassemia and sickle cell disease (SCD) are prototypical Mendelian single gene disorders, both caused by mutations affecting the adult β-globin gene. Despite the apparent genetic simplicity, both disorders display a remarkable spectrum of phenotypic severity and share two major genetic modifiers-α-globin genotype and innate ability to produce fetal hemoglobin (HbF, α
    Language English
    Publishing date 2017
    Publishing country United States
    Document type Journal Article
    ISSN 2214-8019 ; 0065-2598
    ISSN (online) 2214-8019
    ISSN 0065-2598
    DOI 10.1007/978-1-4939-7299-9_2
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  7. Article ; Online: Molecular basis of β thalassemia and potential therapeutic targets.

    Thein, Swee Lay

    Blood cells, molecules & diseases

    2017  Volume 70, Page(s) 54–65

    Abstract: The remarkable phenotypic diversity of β thalassemia that range from severe anemia and transfusion-dependency, to a clinically asymptomatic state exemplifies how a spectrum of disease severity can be generated in single gene disorders. While the genetic ... ...

    Abstract The remarkable phenotypic diversity of β thalassemia that range from severe anemia and transfusion-dependency, to a clinically asymptomatic state exemplifies how a spectrum of disease severity can be generated in single gene disorders. While the genetic basis for β thalassemia, and how severity of the anemia could be modified at different levels of its pathophysiology have been well documented, therapy remains largely supportive with bone marrow transplant being the only cure. Identification of the genetic variants modifying fetal hemoglobin (HbF) production in combination with α globin genotype provide some prediction of disease severity for β thalassemia but generation of a personalized genetic risk score to inform prognosis and guide management requires a larger panel of genetic modifiers yet to be discovered. Nonetheless, genetic studies have been successful in characterizing the key variants and pathways involved in HbF regulation, providing new therapeutic targets for HbF reactivation. BCL11A has been established as a quantitative repressor, and progress has been made in manipulating its expression using genomic and gene-editing approaches for therapeutic benefits. Recent discoveries and understanding in the mechanisms associated with ineffective and abnormal erythropoiesis have also provided additional therapeutic targets, a couple of which are currently being tested in clinical trials.
    MeSH term(s) Animals ; Combined Modality Therapy ; Gene Expression Regulation ; Genetic Association Studies ; Genetic Loci ; Genetic Predisposition to Disease ; Genotype ; Humans ; Mutation ; Phenotype ; beta-Globins/genetics ; beta-Globins/metabolism ; beta-Thalassemia/genetics ; beta-Thalassemia/metabolism ; beta-Thalassemia/therapy
    Chemical Substances beta-Globins
    Language English
    Publishing date 2017-06-20
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Intramural ; Review
    ZDB-ID 1237083-6
    ISSN 1096-0961 ; 1079-9796
    ISSN (online) 1096-0961
    ISSN 1079-9796
    DOI 10.1016/j.bcmd.2017.06.001
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  9. Article ; Online: Sickle cell disease-Unanswered questions and future directions in therapy.

    Thein, Swee Lay / Tisdale, John

    Seminars in hematology

    2018  Volume 55, Issue 2, Page(s) 51–52

    Language English
    Publishing date 2018-05-26
    Publishing country United States
    Document type Editorial
    ZDB-ID 206923-4
    ISSN 1532-8686 ; 0037-1963
    ISSN (online) 1532-8686
    ISSN 0037-1963
    DOI 10.1053/j.seminhematol.2018.05.010
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  10. Article ; Online: The carrier state for sickle cell disease is not completely harmless.

    Xu, Julia Zhe / Thein, Swee Lay

    Haematologica

    2019  Volume 104, Issue 6, Page(s) 1106–1111

    MeSH term(s) Alleles ; Disease Progression ; Exercise ; Hemoglobin, Sickle/genetics ; Heterozygote ; Humans ; Kidney Diseases/etiology ; Kidney Diseases/metabolism ; Kidney Diseases/physiopathology ; Phenotype ; Sickle Cell Trait/blood ; Sickle Cell Trait/complications ; Sickle Cell Trait/diagnosis ; Sickle Cell Trait/genetics
    Chemical Substances Hemoglobin, Sickle
    Language English
    Publishing date 2019-05-16
    Publishing country Italy
    Document type Journal Article ; Review
    ZDB-ID 2333-4
    ISSN 1592-8721 ; 0017-6567 ; 0390-6078
    ISSN (online) 1592-8721
    ISSN 0017-6567 ; 0390-6078
    DOI 10.3324/haematol.2018.206060
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