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Comparative histological analysis of spleens in pediatric patients with hemolytic anemias: Insights into the pathophysiological mechanisms of spleen destruction in sickle cell anemia.
- Source :
-
American journal of hematology [Am J Hematol] 2024 Sep; Vol. 99 (9), pp. 1670-1679. Date of Electronic Publication: 2024 May 22. - Publication Year :
- 2024
-
Abstract
- While sickle cell anemia (SCA) and hereditary spherocytosis (HS) share common features of increased spleen erythrophagocytosis due to increased red blood cell (RBC) turnover, SCA is specifically characterized by susceptibility to infections. In this study, histological lesions in the spleens of pediatric patients with SCA were analyzed, in close correlation with past clinical history and comparatively to HS, healthy and transfused β-thalassemia patients (TDT). An evaluation of red pulp elementary lesions (red pulp fibrosis, iron deposition, number of Gandy-Gamna, and RBC trapping) combined into a severity score was established, as well as B-cell follicles analysis. Quantification on digitalized slides of iron deposition, RBC trapping, and red pulp fibrosis was additionally performed. Spleens from 22 children with SCA, eight with HS, eight with TDT, and three healthy controls (HC) were analyzed. Median age at splenectomy was not different between SCA and HS patients, 6.05 years (range: 4.5-16.0) versus 4.75 (range: 2.2-9.5). Marked heterogeneity was found in SCA spleens in contrast to other conditions. Contrary to previous reports, B-cell follicles were generally preserved in SCA. While RBC trapping was significantly increased in both SCA and HS (compared to TDT and HC), quantitative fibrosis and overall red pulp severity score were significantly increased in SCA spleens compared to other conditions. Moreover, there was an inverse correlation between quantitative fibrosis and number of B-cell follicles, linking these two compartments as well as spleen fibrosis to infectious susceptibility in SCA, potentially through impaired red pulp macrophage scavenging and B-cell subpopulations defects.<br /> (© 2024 The Author(s). American Journal of Hematology published by Wiley Periodicals LLC.)
- Subjects :
- Humans
Child
Adolescent
Male
Female
Child, Preschool
beta-Thalassemia pathology
beta-Thalassemia complications
Splenectomy
Fibrosis
B-Lymphocytes pathology
Anemia, Sickle Cell pathology
Anemia, Sickle Cell complications
Anemia, Sickle Cell blood
Spleen pathology
Spherocytosis, Hereditary pathology
Spherocytosis, Hereditary blood
Subjects
Details
- Language :
- English
- ISSN :
- 1096-8652
- Volume :
- 99
- Issue :
- 9
- Database :
- MEDLINE
- Journal :
- American journal of hematology
- Publication Type :
- Academic Journal
- Accession number :
- 38775210
- Full Text :
- https://doi.org/10.1002/ajh.27374