ArticleAmerican journal of hematology2023
In vivo evaluation of the effect of sickle cell hemoglobin S, C and therapeutic transfusion on erythrocyte metabolism and cardiorenal dysfunction.
Article in American journal of hematology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
23 citing papers in PubMed, 31 citations in OpenAlex.
- Blood-storage duration affects hematological and metabolic profiles in patients with sickle cell disease receiving transfusions.The Journal of clinical investigation · 2025Trial
- Arginine Therapy for Pain in Sickle Cell Disease: A Phase-2 Randomized, Placebo-Controlled Trial.American journal of hematology · 2025Trial
- Functional and multi-omics signatures of mitapivat efficacy upon activation of pyruvate kinase in red blood cells from patients with sickle cell disease.Haematologica · 2024Trial
- Alterations of Plasma Metabolites Associated with Sickle Cell Trait.Clinical journal of the American Society of Nephrology : CJASN · 2026Article
- A predictive model for the risk of death within 30 days in patients with cardiorenal syndrome treated with continuous renal replacement therapy: A retrospective study based on the MIMIC-IV database.Scientific reports · 2026Article
- Iron, arginine, and redox metabolism in peripheral blood mononuclear cells distinguishes sickle cell disease and pulmonary hypertension.HemaSphere · 2026Article
- A mouse model for hemoglobin SC disease recapitulates characteristic human pathologies.Blood advances · 2025Article
- From metabolomics to transfusion-associated immunomodulation.Current opinion in immunology · 2025Review
- Red blood cell metabolism: a window on systems health towards clinical metabolomics.Current opinion in hematology · 2025Review
- Targeting lung heme iron by aerosol hemopexin adminstration in sickle cell disease pulmonary hypertension.Free radical biology & medicine · 2025Article
- Article
- It's in your blood: The impact of age, sex, genetic factors and exposures on stored red blood cell metabolism.Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis · 2024Review
- Complete absence of GLUT1 does not impair human terminal erythroid differentiation.Blood advances · 2024Article
- Metabolic blood profile and response to treatment with the pyruvate kinase activator mitapivat in patients with sickle cell disease.HemaSphere · 2024Article
- Metabolic regulation of erythrocyte development and disorders.Experimental hematology · 2024Article
- Article
- A novel composition of endogenous metabolic modulators improves red blood cell properties in sickle cell disease.EJHaem · 2024Article
- Complete absence of GLUT1 does not impair human terminal erythroid differentiation.bioRxiv : the preprint server for biology · 2024Article
- Metabolic signatures of cardiorenal dysfunction in plasma from sickle cell patients as a function of therapeutic transfusion and hydroxyurea treatment.Haematologica · 2023Article
- Modeling Red Blood Cell Metabolism in the Omics Era.Metabolites · 2023Review
Corrections and comments
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Authors and funding
11 authors at 4 institutions in 1 country.
Funding
Abstract
Despite a wealth of exploratory plasma metabolomics studies in sickle cell disease (SCD), no study to date has evaluate a large and well phenotyped cohort to compare the primary erythrocyte metabolome of hemoglobin SS, SC and transfused AA red blood cells (RBCs) in vivo. The current study evaluates the RBC metabolome of 587 subjects with sickle cell sickle cell disease (SCD) from the WALK-PHaSST clinical cohort. The set includes hemoglobin SS, hemoglobin SC SCD patients, with variable levels of HbA related to RBC transfusion events. Here we explore the modulating effects of genotype, age, sex, severity of hemolysis, and transfusion therapy on sickle RBC metabolism. Results show that RBCs from patients with Hb SS genotypes-compared to AA RBCs from recent transfusion events or SC RBCs-are characterized by significant alterations of RBC acylcarnitines, pyruvate, sphingosine 1-phosphate, creatinine, kynurenine and urate metabolism. Surprisingly, the RBC metabolism of SC RBCs is dramatically different from SS, with all glycolytic intermediates significantly elevated in SS RBCs, with the exception of pyruvate. This result suggests a metabolic blockade at the ATP-generating phosphoenolpyruvate to pyruvate step of glycolysis, which is catalyzed by redox-sensitive pyruvate kinase. Metabolomics, clinical and hematological data were collated in a novel online portal. In conclusion, we identified metabolic signatures of HbS RBCs that correlate with the degree of steady state hemolytic anemia, cardiovascular and renal dysfunction and mortality.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.