biostudies-literatureSissoko AAgence Nationale de la RechercheNHLBI NIH HHSNational Institutes of HealthNIH HHS2267-2278https://www.ebi.ac.uk/biostudies/studies/S-EPMC11560635biostudies-literatureUnknown99(12)In children with sickle cell disease (SCD), splenectomy is immediately beneficial for acute sequestration crises and hypersplenism (ASSC/HyS) but portends a long-term risk of asplenia-related complications. 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-related disease undergoing splenectomy (controls), five human spleens perfused ex vivo with Hb_SS- and Hb_AA-RBC, and quantified abnormal RBC by microscopy, spleen-mimetic 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 <2%, while proAmerican journal of hematologyClearance of pathogenic erythrocytes is maintained despite spleen dysfunction in children with sickle cell disease.PMC11560635RS30J20ANR29_SPLEENMARKR21087HHR01 HL154150Manceau SNdour PAFricot-Monsinjon ADumas LSauvanet ADokmak SHardy DMaitre BEckly APaye FCros JCapito CAllali SAussilhou BCisse ALesurtel MSissoko AWendum DRoussel CMarin MDuverdier CBuffet PDussiot MDembinski JTichit MfalseClearance of pathogenic erythrocytes is maintained despite spleen dysfunction in children with sickle cell disease.In children with sickle cell disease (SCD), splenectomy is immediately beneficial for acute sequestration crises and hypersplenism (ASSC/HyS) but portends a long-term risk of asplenia-related complications. 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-related disease undergoing splenectomy (controls), five human spleens perfused ex vivo with Hb_SS- and Hb_AA-RBC, and quantified abnormal RBC by microscopy, spleen-mimetic 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 <2%, while pro2024-01-01T00:00:00Z2024 Dec2026-06-05T20:55:05.83Z2026-05-21T03:13:01.076ZS-EPMC115606353928696310.1002/ajh.27481