{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T05:04:41Z","timestamp":1789016681649,"version":"build-2803163510"},"reference-count":21,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2024,1,22]],"date-time":"2024-01-22T00:00:00Z","timestamp":1705881600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2024,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Acute splenic sequestration crisis (ASSC) is a potentially life\u2010threatening complication of sickle cell disease (SCD), typically occurring in young patients under 5\u2009years of age, with a median age at first episode of less than 2\u2009years. Because a beneficial effect of hydroxyurea (HU) on spleen perfusion and splenic function has been suspected, we hypothesized that HU treatment might be associated with later onset of ASSC in patients with SCD. To investigate this hypothesis, we analyzed data from the ESCORT\u2010HU study on a large cohort of patients with SCD receiving HU, enrolled between January 2009 and June 2017 with a follow\u2010up of 7309 patient\u2010years of observation. The median age at ASSC of the 14 patients who experienced a first episode of ASSC during the study period was 8.0 [IQR: 5.0\u201324.1] years. The median age at HU initiation was significantly lower in these 14 patients (4.8 [IQR: 3.3\u201318.7] years) compared to the 1664 patients without ASSC (19.9 [8.8\u201333.4] years, <jats:italic>p<\/jats:italic>\u2009=\u2009.0008). These findings suggest that ASSC may occur at an unusually late age in patients receiving HU, possibly reflecting longer preservation of spleen perfusion and function secondary to early initiation of HU. Further studies are needed to better characterize the effects of HU on spleen perfusion\/function and on the occurrence of ASSC in patients with SCD (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/clinicaltrials.gov\">ClinicalTrials.gov<\/jats:ext-link> identifier: NCT02516579; European registry ENCEPP\/SDPP\/10565).<\/jats:p>","DOI":"10.1002\/ajh.27214","type":"journal-article","created":{"date-parts":[[2024,1,22]],"date-time":"2024-01-22T08:29:23Z","timestamp":1705912163000},"page":"555-561","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Hydroxyurea is associated with later onset of acute splenic sequestration crisis in sickle cell disease: Lessons from the European Sickle Cell Disease Cohort\u2014Hydroxyurea (<scp>ESCORT\u2010HU<\/scp>) study"],"prefix":"10.1002","volume":"99","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7068-4530","authenticated-orcid":false,"given":"Slimane","family":"Allali","sequence":"first","affiliation":[{"name":"Department of General Pediatrics and Pediatric Infectious Diseases, Reference Center for Sickle Cell Disease, Necker\u2010Enfants Malades Hospital, Assistance Publique\u2010H\u00f4pitaux de Paris (AP\u2010HP) Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fr\u00e9d\u00e9ric","family":"Galact\u00e9ros","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Sickle Cell Referral Center Henri\u2010Mondor University Hospital\u2010UPEC, AP\u2010HP  Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lena","family":"Oevermann","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology and Hematology Charit\u00e9 University Medicine, Berlin and Berlin Institute of Health  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanna","family":"Cannas","sequence":"additional","affiliation":[{"name":"Hospices Civils de Lyon, H\u00f4pital Edouard Herriot, M\u00e9decine Interne, Centre de R\u00e9f\u00e9rence Constitutif: Syndromes Dr\u00e9panocytaires Majeurs, Thalass\u00e9mies et Autres Pathologies Rares du Globule Rouge et de l'Erythropo\u00ef\u00e8se  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laure","family":"Joseph","sequence":"additional","affiliation":[{"name":"Biotherapy Department, Necker\u2010Enfants Malades Hospital, Assistance Publique\u2010H\u00f4pitaux de Paris (AP\u2010HP) Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gylna","family":"Loko","sequence":"additional","affiliation":[{"name":"Centre Hospitalier Universitaire de Martinique, Centre de R\u00e9f\u00e9rence Constitutif: Syndromes Dr\u00e9panocytaires Majeurs, Thalass\u00e9mies et Autres Pathologies Rares du Globule Rouge et de l'\u00c9rythropo\u00ef\u00e8se des Antilles et de la Guyane  Le Lamentin, Martinique France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Narcisse","family":"Elenga","sequence":"additional","affiliation":[{"name":"Centre Hospitalier de Cayenne, Centre de R\u00e9f\u00e9rence Constitutif: Syndromes Dr\u00e9panocytaires Majeurs, Thalass\u00e9mies et Autres Pathologies Rares du Globule Rouge et de l'Erythropo\u00ef\u00e8se  Cayenne French Guiana"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malika","family":"Benkerrou","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence MCGRE, Service d'H\u00e9matologie\u2010Immunologie, AP\u2010HP, H\u00f4pital Robert Debr\u00e9, Inserm, UMR\u20101123 ECEVE Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maryse","family":"Etienne\u2010Julan","sequence":"additional","affiliation":[{"name":"Unit\u00e9 Transversale de la Dr\u00e9panocytose, Centre de R\u00e9f\u00e9rence Maladies Rares pour la Dr\u00e9panocytose aux Antilles\u2010Guyane, CHU de Pointe\u2010\u00e0\u2010Pitre, Pointe\u2010\u00e0\u2010Pitre  Guadeloupe France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie\u2010Pierre","family":"Castex","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Immunology Hematology Unit Children's University Hospital \u2013 Toulouse University Hospital  Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentine","family":"Brousse","sequence":"additional","affiliation":[{"name":"Department of General Pediatrics and Pediatric Infectious Diseases, Reference Center for Sickle Cell Disease, Necker\u2010Enfants Malades Hospital, Assistance Publique\u2010H\u00f4pitaux de Paris (AP\u2010HP) Universit\u00e9 Paris Cit\u00e9  Paris France"},{"name":"Centre de R\u00e9f\u00e9rence MCGRE, Service d'H\u00e9matologie\u2010Immunologie, AP\u2010HP, H\u00f4pital Robert Debr\u00e9, Inserm, UMR\u20101123 ECEVE Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4900-4753","authenticated-orcid":false,"given":"Mariane","family":"de Montalembert","sequence":"additional","affiliation":[{"name":"Department of General Pediatrics and Pediatric Infectious Diseases, Reference Center for Sickle Cell Disease, Necker\u2010Enfants Malades Hospital, Assistance Publique\u2010H\u00f4pitaux de Paris (AP\u2010HP) Universit\u00e9 Paris Cit\u00e9  Paris 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