ArticleBritish journal of haematology2021
Vasculo-toxic and pro-inflammatory action of unbound haemoglobin, haem and iron in transfusion-dependent patients with haemolytic anaemias.
Article in British journal of haematology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers, 1 of them a synthesis that pooled it.
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Who cites it
34 citing papers in PubMed, 1 synthesis or guideline pooled it, 53 citations in OpenAlex.
- Ferritin and transferrin predict common carotid intima-media thickness in females: a machine-learning informed individual participant data meta-analysis.BMC cardiovascular disorders · 2026Pooled it
- Effects of Sirolimus treatment on patients with β-Thalassemia: Lymphocyte immunophenotype and biological activity of memory CD4Journal of cellular and molecular medicine · 2023Trial
- Article
- Exercise reveals precocious-subclinical cardiovascular and muscular dysfunction in transfusion dependent beta-thalassemia (TDT) patients without cardiac iron overload.Journal of translational medicine · 2026Article
- Effect of Red Blood Cell Transfusion on Inflammatory and Angiogenic Pathways in Patients With Sickle Cell Disease.American journal of hematology · 2026Article
- Liver sinusoidal endothelial cells constitute a major route for hemoglobin clearance.EMBO reports · 2026Article
- Endothelial dysfunction and cardiac damage indicators in patients with β-thalassemia major under iron-chelation therapy.Therapeutic advances in hematology · 2026Article
- Erythropoiesis in health and disease: Distinguishing defective and ineffective erythropoiesis.HemaSphere · 2026Review
- Thalassemia and hypercoagulability.Hematology. American Society of Hematology. Education Program · 2025Review
- Impact of sickle cell anaemia on reproductive health: a narrative review.Reproductive health · 2025Review
- Haploidentical hematopoietic stem cell transplantation for the treatment of congenital dyserythropoietic anemia combined with thalassemia: a report of two cases.Annals of hematology · 2025Article
- Genetic biomarkers and crucial cell subsets of iron metabolism in Beta-Thalassemia: insights from bioinformatics and experimental validation.Annals of hematology · 2025Article
- Elevated serum heme oxygenase-1 in pediatric sickle cell disease: Insights from the SickleGenAfrica Network.HemaSphere · 2025Article
- Clinical features and outcomes of myelodysplastic syndrome patients with iron overload: a single-center retrospective study.European journal of medical research · 2025Article
- Cellular and biochemical heterogeneity contributes to the phenotypic diversity of transfusion-dependent β-thalassemia.Blood advances · 2025Article
- Extracellular vesicles modulate endothelial nitric oxide production in patients with β‑thalassaemia/HbE.Biomedical reports · 2025Article
- Crosstalk Between Sickle Cell Disease and Ferroptosis.International journal of molecular sciences · 2025Review
- Endothelial activation in thromboangiitis obliterans: mechanisms and therapeutic horizons.Frontiers in immunology · 2025Review
- The Redox Process in Red Blood Cells: Balancing Oxidants and Antioxidants.Antioxidants (Basel, Switzerland) · 2024Review
- News on sickle cell disease: Heme-driven disordered erythropoiesis.HemaSphere · 2024Article
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Authors and funding
17 authors at 9 institutions in 7 countries.
Funding
Abstract
Increasing evidence suggests that free haem and iron exert vasculo-toxic and pro-inflammatory effects by activating endothelial and immune cells. In the present retrospective study, we compared serum samples from transfusion-dependent patients with β-thalassaemia major and intermedia, hereditary spherocytosis and sickle cell disease (SCD). Haemolysis, transfusions and ineffective erythropoiesis contribute to haem and iron overload in haemolytic patients. In all cohorts we observed increased systemic haem and iron levels associated with scavenger depletion and toxic 'free' species formation. Endothelial dysfunction, oxidative stress and inflammation markers were significantly increased compared to healthy donors. In multivariable logistic regression analysis, oxidative stress markers remained significantly associated with both haem- and iron-related parameters, while soluble vascular cell adhesion molecule 1 (sVCAM-1), soluble endothelial selectin (sE-selectin) and tumour necrosis factor α (TNFα) showed the strongest association with haem-related parameters and soluble intercellular adhesion molecule 1 (sICAM-1), sVCAM-1, interleukin 6 (IL-6) and vascular endothelial growth factor (VEGF) with iron-related parameters. While hereditary spherocytosis was associated with the highest IL-6 and TNFα levels, β-thalassaemia major showed limited inflammation compared to SCD. The sVCAM1 increase was significantly lower in patients with SCD receiving exchange compared to simple transfusions. The present results support the involvement of free haem/iron species in the pathogenesis of vascular dysfunction and sterile inflammation in haemolytic diseases, irrespective of the underlying haemolytic mechanism, and highlight the potential therapeutic benefit of iron/haem scavenging therapies in these conditions.
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