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The significance of spleen size in children with sickle cell anemia.
Am J Hematol. 2022 12; 97(12):1520-1528.AJ

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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Authors+Show Affiliations

Nardo-Marino A
Danish Centre for Haemoglobinopathies, Department of Haematology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. Department of Immunology and Microbiology, Centre for Medical Parasitology, University of Copenhagen, Copenhagen, Denmark. Department of Haematological Medicine, King's College Hospital, London, United Kingdom. Comprehensive Cancer Centre, School of Cancer and Pharmaceutical Sciences, King's College London, London, United Kingdom.
Glenthøj A
Danish Centre for Haemoglobinopathies, Department of Haematology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Brewin JN
Department of Haematological Medicine, King's College Hospital, London, United Kingdom. Comprehensive Cancer Centre, School of Cancer and Pharmaceutical Sciences, King's College London, London, United Kingdom.
Petersen J
Danish Centre for Haemoglobinopathies, Department of Haematology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Braunstein TH
Core Facility for Integrated Microscopy, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark.
Kurtzhals JAL
Department of Immunology and Microbiology, Centre for Medical Parasitology, University of Copenhagen, Copenhagen, Denmark. Department of Clinical Microbiology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Williams TN
KEMRI-Wellcome Trust Research Programme, Kilifi, Kenya. Department of Surgery and Cancer, Institute of Global Health Innovation, Imperial College London, London, United Kingdom.
Rees DC
Department of Haematological Medicine, King's College Hospital, London, United Kingdom. Comprehensive Cancer Centre, School of Cancer and Pharmaceutical Sciences, King's College London, London, United Kingdom.

MeSH

ChildChild, PreschoolHumansSpleenAnemia, Sickle CellSplenomegalyHydroxyureaErythrocyte Count

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

36054667

Citation

Nardo-Marino, Amina, et al. "The Significance of Spleen Size in Children With Sickle Cell Anemia." American Journal of Hematology, vol. 97, no. 12, 2022, pp. 1520-1528.
Nardo-Marino A, Glenthøj A, Brewin JN, et al. The significance of spleen size in children with sickle cell anemia. Am J Hematol. 2022;97(12):1520-1528.
Nardo-Marino, A., Glenthøj, A., Brewin, J. N., Petersen, J., Braunstein, T. H., Kurtzhals, J. A. L., Williams, T. N., & Rees, D. C. (2022). The significance of spleen size in children with sickle cell anemia. American Journal of Hematology, 97(12), 1520-1528. https://doi.org/10.1002/ajh.26703
Nardo-Marino A, et al. The Significance of Spleen Size in Children With Sickle Cell Anemia. Am J Hematol. 2022;97(12):1520-1528. PubMed PMID: 36054667.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The significance of spleen size in children with sickle cell anemia. AU - Nardo-Marino,Amina, AU - Glenthøj,Andreas, AU - Brewin,John N, AU - Petersen,Jesper, AU - Braunstein,Thomas H, AU - Kurtzhals,Jørgen A L, AU - Williams,Thomas N, AU - Rees,David C, Y1 - 2022/09/27/ PY - 2022/08/21/revised PY - 2022/05/31/received PY - 2022/08/23/accepted PY - 2022/9/3/pubmed PY - 2022/11/11/medline PY - 2022/9/2/entrez SP - 1520 EP - 1528 JF - American journal of hematology JO - Am J Hematol VL - 97 IS - 12 N2 - 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. SN - 1096-8652 UR - https://www.unboundmedicine.com/medline/citation/36054667/The_significance_of_spleen_size_in_children_with_sickle_cell_anemia_ DB - PRIME DP - Unbound Medicine ER -