ArticleAmerican journal of hematology2022
The significance of spleen size in children with sickle cell anemia.
Article in American journal of hematology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
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Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it, 18 citations in OpenAlex.
- Genetic Variation and Sickle Cell Disease Severity: A Systematic Review and Meta-Analysis.JAMA network open · 2023Pooled it
- Prevalence and clinical correlates of splenomegaly in sickle cell disease patients: An observational study in Western India.Medical journal, Armed Forces India · 2025Article
- Infections in sickle cell disease.Haematologica · 2025Review
- From early development to maturity: a phenotypic analysis of the Townes sickle cell disease mice.Biology open · 2025Article
- Improved Preprocedure Hematocrit in Chronic RBC Exchange Patients Following Splenectomy: A Case Report.Case reports in hematology · 2025Article
- Hematopoietic effects of Fufang E'jiao Jiang revealed by microbiome, metabolome and transcriptome analyses: a multi-omics strategy.Frontiers in immunology · 2025Article
- Clearance of pathogenic erythrocytes is maintained despite spleen dysfunction in children with sickle cell disease.American journal of hematology · 2024Article
- Article
- Clinical and laboratory factors associated with splenic dysfunction among patients with sickle cell disease in a malaria endemic region.Transactions of the Royal Society of Tropical Medicine and Hygiene · 2023Article
- Early splenectomy in a large cohort of children with sickle cell anemia: risks and consequences.Haematologica · 2023Article
- Role of Macrophages in Sickle Cell Disease Erythrophagocytosis and Erythropoiesis.International journal of molecular sciences · 2023Review
- Microfluidic study of retention and elimination of abnormal red blood cells by human spleen with implications for sickle cell disease.Proceedings of the National Academy of Sciences of the United States of America · 2023Article
- The significance of spleen size in children with sickle cell anemia.American journal of hematology · 2022Article
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 3 countries.
Funding
Abstract
It is well established that splenic dysfunction occurs in early childhood in sickle cell anemia (SCA), although the determinants and consequences of splenic injury are not fully understood. In this study, we examined spleen size and splenic function in 100 children with SCA aged 0-16 years at King's College Hospital in London. Spleen size was assessed by abdominal ultrasound (US) and splenic function by pitted red blood cells (PIT counts). In our cohort, 5.6% of children aged 6-10 years and 19.4% of children aged 11-16 years had no visible spleen on US (autosplenectomy). Splenomegaly was common in all age groups, with 28% of children overall having larger spleens than the average for their age. Only one child had a PIT count suggesting preserved splenic function. We found no correlation between hemoglobin F levels and spleen size, nor was there any difference in spleen size between children treated with or without hydroxyurea. Although there was a trend toward increased spleen length in children with co-inherited α-thalassemia, this did not reach statistical significance. Finally, we found a strong association between erythrocyte deformability measured with oxygen gradient ektacytometry, spleen size, and PIT counts. In conclusion, our results do not agree with the general perception that most children with SCA undergo autosplenectomy within the first decade of life and indicate that loss of erythrocyte deformability contributes to loss of splenic filtration capacity in SCA, as well as phenotypical variations in spleen size.
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