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We retrieved peripheral and splenic red blood cells (RBCs) from 17 SCD children\/teenagers undergoing partial splenectomy for ASSC\/HyS, 12 adult subjects without RBC\u2010related disease undergoing splenectomy (controls), five human spleens perfused ex vivo with Hb<jats:sub>SS<\/jats:sub>\u2010 and Hb<jats:sub>AA<\/jats:sub>\u2010RBC, and quantified abnormal RBC by microscopy, spleen\u2010mimetic RBC filtration, and adhesion assays. Spleens were analyzed by immunohistochemistry and transmission electron microscopy (TEM). In circulating blood of SCD and control subjects, dysmorphic (elongated\/spherocytic) RBCs were &lt;2%, while proportions of pocked\u2010RBC were 4.3\u2010fold higher in SCD children than in controls. Compared to controls, splenic RBCs were more frequently dysmorphic (29.3% vs. 0.4%), stiffer (42.2% vs. 12.4%), and adherent (206 vs. 22 adherent RBC\/area) in SCD subjects. By TEM, both polymer\u2010containing and homogenous RBC contributed to spleen congestion, resulting in 3.8\u2010fold higher RBC population density in SCD spleens than in control spleens, predominantly in the cords. Perfused spleens with normal function displayed similar congestion and retention of dysmorphic RBC as SCD spleens. The population density of active macrophages was similar in SCD and control spleens, with a relative deficit in phagocytosis of polymer\u2010containing RBC. Despite the existence of hyposplenism, splenectomy in SCD children removes an organ that still efficiently filters out potentially pathogenic altered RBC. Innovative treatments allowing fine\u2010tuned reduction of RBC retention would alleviate spleen congestion, the major pathogenic process in ASSC\/HyS, while preserving spleen protective functions for the future.<\/jats:p>","DOI":"10.1002\/ajh.27481","type":"journal-article","created":{"date-parts":[[2024,9,17]],"date-time":"2024-09-17T17:55:11Z","timestamp":1726595711000},"page":"2267-2278","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Clearance of pathogenic erythrocytes is maintained despite spleen dysfunction in children with sickle cell disease"],"prefix":"10.1002","volume":"99","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9986-0494","authenticated-orcid":false,"given":"Abdoulaye","family":"Sissoko","sequence":"first","affiliation":[{"name":"Universit\u00e9 Paris Cit\u00e9, Inserm, BIGR  Paris 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