{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Sissoko A"],"funding":["Agence Nationale de la Recherche","NHLBI NIH HHS","National Institutes of Health","NIH HHS"],"pagination":["2267-2278"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC11560635"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["99(12)"],"pubmed_abstract":["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<sub>SS</sub>- and Hb<sub>AA</sub>-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 pro"],"journal":["American journal of hematology"],"pubmed_title":["Clearance of pathogenic erythrocytes is maintained despite spleen dysfunction in children with sickle cell disease."],"pmcid":["PMC11560635"],"funding_grant_id":["RS30J20ANR29_SPLEENMARK","R21087HH","R01 HL154150"],"pubmed_authors":["Manceau S","Ndour PA","Fricot-Monsinjon A","Dumas L","Sauvanet A","Dokmak S","Hardy D","Maitre B","Eckly A","Paye F","Cros J","Capito C","Allali S","Aussilhou B","Cisse A","Lesurtel M","Sissoko A","Wendum D","Roussel C","Marin M","Duverdier C","Buffet P","Dussiot M","Dembinski J","Tichit M"],"additional_accession":[]},"is_claimable":false,"name":"Clearance of pathogenic erythrocytes is maintained despite spleen dysfunction in children with sickle cell disease.","description":"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<sub>SS</sub>- and Hb<sub>AA</sub>-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 pro","dates":{"release":"2024-01-01T00:00:00Z","publication":"2024 Dec","modification":"2026-06-05T20:55:05.83Z","creation":"2026-05-21T03:13:01.076Z"},"accession":"S-EPMC11560635","cross_references":{"pubmed":["39286963"],"doi":["10.1002/ajh.27481"]}}