ArticleHaematologica2021
Fetal hemoglobin rescues ineffective erythropoiesis in sickle cell disease.
Article in Haematologica, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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Who cites it
40 citing papers in PubMed, 59 citations in OpenAlex.
- Severe inflammation and lineage skewing are associated with poor engraftment of engineered hematopoietic stem cells in patients with sickle cell disease.Nature communications · 2025Trial
- Human erythroid models of sickle cell disease.HemaSphere · 2026Review
- Premature senescence impairs hematopoietic stem cell function during sickle cell disease in mice and humans.Science translational medicine · 2026Article
- Daprodustat augments red cell and γ-globin production in models of sickle cell disease.Blood red cells & iron · 2026Article
- Xinnaoxin tablets ameliorate high-altitude polycythemia-associated cardiac injury by regulating the NF-κB, MAPK, and PI3K/AKT signaling pathways.Frontiers in pharmacology · 2026Article
- Erythropoiesis in health and disease: Distinguishing defective and ineffective erythropoiesis.HemaSphere · 2026Review
- A mouse model for hemoglobin SC disease recapitulates characteristic human pathologies.Blood advances · 2025Article
- When Blood Disorders Meet Cancer: Uncovering the Oncogenic Landscape of Sickle Cell Disease.Journal of clinical medicine · 2025Review
- Selective AMPKβ1 activation induces fetal hemoglobin in human erythroid cells and sickle cell mice via the noncanonical NRF2 pathway.Science advances · 2025Article
- Optimizing CRISPR methodology for precise gene editing in the erythroid cell line BEL-A with high efficiency generation of a sickle cell anemia model.Haematologica · 2025Article
- To pursue gene therapy or not? Is it feasible after graft failure in allogeneic hematopoietic cell transplant recipients.Blood advances · 2025Review
- Article
- Clinical manifestations of sickle cell disease in Africa and its association with foetal haemoglobin parameters.Communications medicine · 2025Article
- BCL11A-deficient human erythropoiesis is impaired in vitro and after xenotransplantation into mice.Blood advances · 2025Article
- Article
- Reduced GATA1 levels are associated with ineffective erythropoiesis in sickle cell anemia.Haematologica · 2025Article
- From old to new: Repurposed drugs in the battle towards curing sickle cell disease.British journal of haematology · 2025Article
- Adolescents and young adults with sickle cell disease exhibit accelerated aging with elevated T-cell p16Aging · 2024Article
- Article
- Whole Blood Transcriptome Analysis in Congenital Anemia Patients.International journal of molecular sciences · 2024Article
Corrections and comments
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Authors and funding
14 authors at 6 institutions in 2 countries.
Funding
Abstract
While ineffective erythropoiesis has long been recognized as a key contributor to anemia in thalassemia, its role in anemia of sickle cell disease (SCD) has not been critically explored. Using in vitro and in vivo derived human erythroblasts we assessed the extent of ineffective erythropoiesis in SCD. Modeling the bone marrow hypoxic environment, we found that hypoxia induces death of sickle erythroblasts starting at the polychromatic stage, positively selecting cells with high levels of fetal hemoglobin (HbF). Cell death was associated with cytoplasmic sequestration of heat shock protein 70 and was rescued by induction of HbF synthesis. Importantly, we document that in the bone marrow of SCD patients similar cell loss occurs during the final stages of terminal differentiation. Our study provides evidence for ineffective erythropoiesis in SCD and highlights an anti-apoptotic role for HbF during the terminal stages of erythroid differentiation. These findings imply that the beneficial effect on anemia of increased HbF levels is not only due to the increased life span of red cells but also a consequence of decreased ineffective erythropoiesis.
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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.