{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T03:15:35Z","timestamp":1770779735856,"version":"3.50.0"},"reference-count":31,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2024,5,22]],"date-time":"2024-05-22T00:00:00Z","timestamp":1716336000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2024,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>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 \u03b2\u2010thalassemia patients (TDT). An evaluation of red pulp elementary lesions (red pulp fibrosis, iron deposition, number of Gandy\u2013Gamna, and RBC trapping) combined into a severity score was established, as well as B\u2010cell 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\u2009years (range: 4.5\u201316.0) versus 4.75 (range: 2.2\u20139.5). Marked heterogeneity was found in SCA spleens in contrast to other conditions<jats:italic>.<\/jats:italic> Contrary to previous reports, B\u2010cell 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\u2010cell follicles, linking these two compartments as well as spleen fibrosis to infectious susceptibility in SCA, potentially through impaired red pulp macrophage scavenging and B\u2010cell subpopulations defects.<\/jats:p>","DOI":"10.1002\/ajh.27374","type":"journal-article","created":{"date-parts":[[2024,5,22]],"date-time":"2024-05-22T10:26:35Z","timestamp":1716373595000},"page":"1670-1679","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Comparative histological analysis of spleens in pediatric patients with hemolytic anemias: Insights into the pathophysiological mechanisms of spleen destruction in sickle cell anemia"],"prefix":"10.1002","volume":"99","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4984-754X","authenticated-orcid":false,"given":"Valentine","family":"Brousse","sequence":"first","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence MCGRE, Service d'H\u00e9mato\u2010Immunologie H\u00f4pital Universitaire Robert Debr\u00e9, APHP  Paris France"},{"name":"Biologie Int\u00e9gr\u00e9e du Globule Rouge, Unit\u00e9 Mixte de Recherche S1134, INSERM Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sara","family":"El Hoss","sequence":"additional","affiliation":[{"name":"Red Cell Hematology Lab, School of Cancer &amp; Pharmaceutical Sciences King's College London, Rayne Institute  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pierre","family":"Isnard","sequence":"additional","affiliation":[{"name":"D\u00e9partement d'Anatomopathologie, H\u00f4pitaux Universitaires Necker Enfants\u2010Malades et Robert Debr\u00e9, APHP Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sandrine","family":"Laurance","sequence":"additional","affiliation":[{"name":"Biologie Int\u00e9gr\u00e9e du Globule Rouge, Unit\u00e9 Mixte de Recherche S1134, INSERM Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Camille","family":"Lambert","sequence":"additional","affiliation":[{"name":"D\u00e9partement d'Anatomopathologie, H\u00f4pitaux Universitaires Necker Enfants\u2010Malades et Robert Debr\u00e9, APHP Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Liza","family":"Ali","sequence":"additional","affiliation":[{"name":"Chirurgie Visc\u00e9rale, H\u00f4pital Universitaire Robert Debr\u00e9, APHP Universit\u00e9 Paris Cit\u00e9  Paris 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Debr\u00e9, APHP  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florence","family":"Missud","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence MCGRE, Service d'H\u00e9mato\u2010Immunologie H\u00f4pital Universitaire Robert Debr\u00e9, APHP  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laurent","family":"Holvoet","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence MCGRE, Service d'H\u00e9mato\u2010Immunologie H\u00f4pital Universitaire Robert Debr\u00e9, APHP  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ghislaine","family":"Ithier","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence MCGRE, Service d'H\u00e9mato\u2010Immunologie H\u00f4pital Universitaire Robert Debr\u00e9, APHP  Paris 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Universite Paris Cite  Paris France"},{"name":"Laboratory of Cellular and Molecular Mechanisms of Hematological Disorders and Therapeutical Implications Universite Paris Cite, Imagine Institute, Inserm U1163  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6318-0227","authenticated-orcid":false,"given":"Leon","family":"Tshilolo","sequence":"additional","affiliation":[{"name":"Centre Hospitalier Monkol\u00e9 Monkol\u00e9  Kinshasa Democratic Republic of the Congo"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacques","family":"Diebold","sequence":"additional","affiliation":[{"name":"D\u00e9partement d'Anatomopathologie, H\u00f4pitaux Universitaires Necker Enfants\u2010Malades et Robert Debr\u00e9, APHP Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thierry Jo","family":"Molina","sequence":"additional","affiliation":[{"name":"D\u00e9partement 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