{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T03:24:29Z","timestamp":1790220269122,"version":"4.1.0"},"reference-count":27,"publisher":"American Society of Hematology","issue":"3","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,7,21]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Splenectomy is effective in \u223c70% to 80% of pediatric chronic immune thrombocytopenia (cITP) cases, and few data exist about it in autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES). Because of the irreversibility of the procedure and the lack of predictions regarding long-term outcomes, the decision to undertake splenectomy is difficult in children. We report here factors associated with splenectomy outcomes from the OBS\u2019CEREVANCE cohort, which prospectively includes French children with autoimmune cytopenia (AIC) since 2004. The primary outcome was failure-free survival (FFS), defined as the time from splenectomy to the initiation of a second-line treatment (other than steroids and intravenous immunoglobulins) or death. We included 161 patients (cITP, n = 120; AIHA, n = 19; ES, n = 22) with a median (minimum-maximum) follow-up of 6.8 years (1.0-33.3) after splenectomy. AIC subtype was not associated with FFS. We found that immunopathological manifestations (IMs) were strongly associated with unfavorable outcomes. Diagnosis of an IM before splenectomy was associated with a lower FFS (hazard ratio [HR], 0.39; 95% confidence interval [CI], 0.21-0.72, P = .003, adjusted for AIC subtype). Diagnosis of an IM at any timepoint during follow-up was associated with an even lower FFS (HR, 0.22; 95% CI, 0.12-0.39; P = 2.8 \u00d7 10\u22127, adjusted for AIC subtype) as well as with higher risk of recurrent or severe bacterial infections and thrombosis. In conclusion, our results support the search for associated IMs when considering a splenectomy to refine the risk-benefit ratio. After the procedure, monitoring IMs helps to identify patients with higher risk of unfavorable outcomes.<\/jats:p>","DOI":"10.1182\/blood.2022015508","type":"journal-article","created":{"date-parts":[[2022,4,20]],"date-time":"2022-04-20T18:52:10Z","timestamp":1650480730000},"page":"253-261","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":15,"title":["Determinants of long-term outcomes of splenectomy in pediatric autoimmune cytopenias"],"prefix":"10.1182","volume":"140","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9412-333X","authenticated-orcid":false,"given":"Thomas","family":"Pincez","sequence":"first","affiliation":[{"name":"1Centre de R\u00e9f\u00e9rence National des Cytop\u00e9nies Auto-immunes de l\u2019Enfant (CEREVANCE), Bordeaux, France;"},{"name":"2Division of Pediatric Hematology-Oncology, Charles-Bruneau Cancer Center, Department of Pediatrics, Sainte-Justine University Hospital, Universit\u00e9 de Montr\u00e9al, Montr\u00e9al, QC, Canada;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0231-4460","authenticated-orcid":false,"given":"Nathalie","family":"Aladjidi","sequence":"additional","affiliation":[{"name":"1Centre de R\u00e9f\u00e9rence National des Cytop\u00e9nies Auto-immunes de l\u2019Enfant (CEREVANCE), Bordeaux, France;"},{"name":"3Pediatric Haemato-Immunology, CIC1401, INSERM CICP, Bordeaux University Hospital, Bordeaux, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0384-6370","authenticated-orcid":false,"given":"S\u00e9bastien","family":"H\u00e9ritier","sequence":"additional","affiliation":[{"name":"4Pediatric Oncology Immunology Hematology Unit, Armand-Trousseau University Hospital, AP-HP, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Garnier","sequence":"additional","affiliation":[{"name":"5Institute of Pediatric Hematology and Oncology, Hospices Civils de Lyon, Lyon, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mony","family":"Fahd","sequence":"additional","affiliation":[{"name":"6Pediatric Hematology Unit, Robert-Debr\u00e9 University Hospital, AP-HP, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wadih","family":"Abou Chahla","sequence":"additional","affiliation":[{"name":"7Department of Pediatric Hematology, Jeanne de Flandre Hospital, Lille University Hospital, Lille, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helder","family":"Fernandes","sequence":"additional","affiliation":[{"name":"1Centre de R\u00e9f\u00e9rence National des Cytop\u00e9nies Auto-immunes de l\u2019Enfant (CEREVANCE), Bordeaux, France;"},{"name":"3Pediatric Haemato-Immunology, CIC1401, INSERM CICP, Bordeaux University Hospital, Bordeaux, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Dichamp","sequence":"additional","affiliation":[{"name":"8Pediatric C-Sickle Cell center-Hemato-Oncology, Maison de la Femme, de la M\u00e8re et de l'Enfant, CHU de Martinique, Fort-de-France, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phane","family":"Ducassou","sequence":"additional","affiliation":[{"name":"1Centre de R\u00e9f\u00e9rence National des Cytop\u00e9nies Auto-immunes de l\u2019Enfant (CEREVANCE), Bordeaux, France;"},{"name":"3Pediatric Haemato-Immunology, CIC1401, INSERM CICP, Bordeaux University Hospital, Bordeaux, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6919-8383","authenticated-orcid":false,"given":"Marl\u00e8ne","family":"Pasquet","sequence":"additional","affiliation":[{"name":"9Pediatric Oncology Immunology Hematology Unit, Children\u2019s University Hospital, Toulouse, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Bayart","sequence":"additional","affiliation":[{"name":"10Pediatric Hematology Unit, Rennes University Hospital, Rennes, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Despina","family":"Moshous","sequence":"additional","affiliation":[{"name":"11Pediatric Hematology-Immunology and Rheumatology Department, H\u00f4pital Necker-Enfants Malades, AP-HP, Paris, France;"},{"name":"12Paris University, Paris; INSERM UMR 1163, Institut Imagine, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3403-3842","authenticated-orcid":false,"given":"Nathalie","family":"Cheikh","sequence":"additional","affiliation":[{"name":"13Department of Pediatric Hematology-Oncology, Besan\u00e7on University Hospital, Besan\u00e7on, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Paillard","sequence":"additional","affiliation":[{"name":"14Department of Pediatric Hematology and Oncology, Hautepierre University Hospital, Strasbourg, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Plantaz","sequence":"additional","affiliation":[{"name":"15Pediatric Hematology-Oncology Department, Grenoble University Hospital, Grenoble, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0318-3044","authenticated-orcid":false,"given":"Eric","family":"Jeziorski","sequence":"additional","affiliation":[{"name":"16Pediatric Oncology Hematology Unit, Arnaud de Villeneuve University Hospital, Montpellier, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Thomas","sequence":"additional","affiliation":[{"name":"17Pediatric Hematology Unit, Nantes University 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