{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T03:15:04Z","timestamp":1791342904623,"version":"4.3.1"},"reference-count":78,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2014,5,26]],"date-time":"2014-05-26T00:00:00Z","timestamp":1401062400000},"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":["Br J Haematol"],"published-print":{"date-parts":[[2014,7]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The spleen has a combined function of immune defence and quality control of senescent or altered red cells. It is the first organ injured in sickle cell anaemia (<jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>) with evidence of hyposplenism present before 12\u00a0months in the majority of children. Repeated splenic vaso\u2010occlusion leads to fibrosis and progressive atrophy of the organ (autosplenectomy), which is generally complete by 5\u00a0years in <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>. The precise sequence of pathogenic events leading to hyposplenism is unknown. Splenic injury is generally silent and progressive. It can be clinically overt with acute splenic sequestration of red cells, an unpredictable and life\u2010threatening complication in infants. Splenomegaly, with or without hypersplenism, can also occur and can coexist with loss of function. Hyposplenism increases the susceptibility of <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content> children to infection with encapsulated bacteria, which is notably reduced by penicillin prophylaxis and immunization. Whether hyposplenism indirectly increases the risk of vaso\u2010occlusion or other circulatory complications remains to be determined.<\/jats:p>","DOI":"10.1111\/bjh.12950","type":"journal-article","created":{"date-parts":[[2014,5,26]],"date-time":"2014-05-26T05:57:08Z","timestamp":1401083828000},"page":"165-176","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":248,"title":["The spleen and sickle cell disease: the sick(led) spleen"],"prefix":"10.1111","volume":"166","author":[{"given":"Valentine","family":"Brousse","sequence":"first","affiliation":[{"name":"Department of Paediatrics Reference Centre for Sickle Cell Disease H\u00f4pital Universitaire Necker\u2010Enfants Malades APHP Paris France"},{"name":"Universit\u00e9 Paris Descartes Paris France"},{"name":"Laboratory of Excellence GR\u2010Ex Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Buffet","sequence":"additional","affiliation":[{"name":"Laboratory of Excellence GR\u2010Ex Paris France"},{"name":"Centre d'Immunologie et des Maladies Infectieuses de Paris CIMI\u2010PARIS U 1135 INSERM\/UPMC Universit\u00e9 Paris VI Paris France"},{"name":"Service de Parasitologie AP\u2010HP H\u00f4pital Piti\u00e9\u2010Salp\u00eatri\u00e8re Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Rees","sequence":"additional","affiliation":[{"name":"Department of Paediatric Haematology King's College Hospital NHS Foundation Trust King's Health Partners Denmark Hill London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,5,26]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)80623-7"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00296-010-1632-x"},{"key":"e_1_2_14_4_1","doi-asserted-by":"publisher","DOI":"10.5144\/0256-4947.1998.217"},{"key":"e_1_2_14_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197710272971701"},{"key":"e_1_2_14_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/tmi.12056"},{"key":"e_1_2_14_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(97)70284-1"},{"key":"e_1_2_14_8_1","doi-asserted-by":"publisher","DOI":"10.1159\/000205060"},{"key":"e_1_2_14_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-197103000-00002"},{"key":"e_1_2_14_10_1","first-page":"677","article-title":"Reversible splenic hypofunction in hypertransfused children with homozygous sickle cell disease","volume":"85","author":"Barrios N.J.","year":"1993","journal-title":"Journal of the National Medical Association"},{"key":"e_1_2_14_11_1","volume-title":"Le Spleen de Paris","author":"Baudelaire C.","year":"1869"},{"key":"e_1_2_14_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2011.08999.x"},{"key":"e_1_2_14_13_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1994.02170080026005"},{"key":"e_1_2_14_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-10-4094"},{"key":"e_1_2_14_15_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0023164"},{"key":"e_1_2_14_16_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1997.d01-1974.x"},{"key":"e_1_2_14_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/aja.1001340404"},{"key":"e_1_2_14_18_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-04-210112"},{"key":"e_1_2_14_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-10-312801"},{"key":"e_1_2_14_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(10)61493-6"},{"key":"e_1_2_14_21_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1935.02760070020005"},{"key":"e_1_2_14_22_1","doi-asserted-by":"crossref","first-page":"1166","DOI":"10.1152\/ajplegacy.1974.227.5.1166","article-title":"Active and passive release of blood from canine spleen and small intestine","volume":"227","author":"Donald D.E.","year":"1974","journal-title":"American Journal of Physiology"},{"key":"e_1_2_14_23_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.27.16686"},{"key":"e_1_2_14_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(85)80125-6"},{"key":"e_1_2_14_25_1","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1182\/blood.V81.4.1102.bloodjournal8141102","article-title":"Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia","volume":"81","author":"Ferster A.","year":"1993","journal-title":"Blood"},{"key":"e_1_2_14_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2007.12.022"},{"key":"e_1_2_14_27_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198606193142501"},{"key":"e_1_2_14_28_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa035477"},{"key":"e_1_2_14_29_1","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1160\/nukmed-0063","article-title":"Scintigraphy with 99mTc\u2010labeled heat\u2010altered erythrocytes in diagnosing hyposplenia: prospective comparison to 99mTc\u2010labeled colloids and colour\u2010coded duplex ultrasonography","volume":"46","author":"Gotthardt M.","year":"2007","journal-title":"Nuklearmedizin"},{"key":"e_1_2_14_30_1","doi-asserted-by":"publisher","DOI":"10.1159\/000335216"},{"key":"e_1_2_14_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.exphem.2006.09.013"},{"key":"e_1_2_14_32_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198206173062402"},{"key":"e_1_2_14_33_1","first-page":"CD003427","article-title":"Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease","volume":"9","author":"Hirst C.","year":"2012","journal-title":"Cochrane Database Systematic Review"},{"key":"e_1_2_14_34_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM196909042811002"},{"key":"e_1_2_14_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tmaid.2009.01.002"},{"key":"e_1_2_14_36_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-010-2587-4"},{"key":"e_1_2_14_37_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21475"},{"key":"e_1_2_14_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)80529-3"},{"key":"e_1_2_14_39_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-005-0061-5"},{"key":"e_1_2_14_40_1","doi-asserted-by":"publisher","DOI":"10.1086\/599834"},{"key":"e_1_2_14_41_1","doi-asserted-by":"crossref","first-page":"2238","DOI":"10.1182\/blood.V85.8.2238.bloodjournal8582238","article-title":"Functional asplenia in hemoglobin SC disease","volume":"85","author":"Lane P.A.","year":"1995","journal-title":"Blood"},{"key":"e_1_2_14_42_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.311.7020.1600"},{"key":"e_1_2_14_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2009.02.016"},{"key":"e_1_2_14_44_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-07-233528"},{"key":"e_1_2_14_45_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-01-265249"},{"key":"e_1_2_14_46_1","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.10.4664"},{"key":"e_1_2_14_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2010.11.025"},{"key":"e_1_2_14_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjmed.2012.12.016"},{"key":"e_1_2_14_49_1","first-page":"288","article-title":"Cerebrovascular accidents in sickle cell disease: rates and risk factors","volume":"91","author":"Ohene\u2010Frempong K.","year":"1998","journal-title":"Blood"},{"key":"e_1_2_14_50_1","first-page":"CD003425","article-title":"Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease","volume":"5","author":"Owusu\u2010Ofori S.","year":"2013","journal-title":"Cochrane Database Systematic Review"},{"key":"e_1_2_14_51_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1005565"},{"key":"e_1_2_14_52_1","doi-asserted-by":"publisher","DOI":"10.1097\/INF.0b013e3182a11808"},{"key":"e_1_2_14_53_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM196910232811703"},{"key":"e_1_2_14_54_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197008132830703"},{"key":"e_1_2_14_55_1","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1542\/peds.76.3.392","article-title":"Developmental pattern of splenic dysfunction in sickle cell disorders","volume":"76","author":"Pearson H.A.","year":"1985","journal-title":"Pediatrics"},{"key":"e_1_2_14_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2011.08.011"},{"key":"e_1_2_14_57_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2011.08806.x"},{"key":"e_1_2_14_58_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-3468(92)90315-X"},{"key":"e_1_2_14_59_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-07-233700"},{"key":"e_1_2_14_60_1","doi-asserted-by":"publisher","DOI":"10.1128\/CMR.00048-09"},{"key":"e_1_2_14_61_1","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0b013e318155abb9"},{"key":"e_1_2_14_62_1","doi-asserted-by":"publisher","DOI":"10.1097\/CCM.0b013e3182416f23"},{"key":"e_1_2_14_63_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-04-278747"},{"key":"e_1_2_14_64_1","doi-asserted-by":"publisher","DOI":"10.1002\/eji.201343577"},{"key":"e_1_2_14_65_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2009.07771.x"},{"key":"e_1_2_14_66_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-03-146779"},{"key":"e_1_2_14_67_1","first-page":"251","volume-title":"The Complete Spleen: A Handbook of Structure, Function, and Clinical Disorders","author":"Serjeant G.R.","year":"2001"},{"key":"e_1_2_14_68_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjmed.2006.08.015"},{"key":"e_1_2_14_69_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1999.1331c.x"},{"key":"e_1_2_14_70_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.10937"},{"key":"e_1_2_14_71_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.56.10.765"},{"key":"e_1_2_14_72_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2005.05767.x"},{"key":"e_1_2_14_73_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2001.119590"},{"key":"e_1_2_14_74_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(11)60355-3"},{"key":"e_1_2_14_75_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-01-0346"},{"key":"e_1_2_14_76_1","volume-title":"Guidelines for the treatment of malaria","author":"WHO","year":"2010"},{"key":"e_1_2_14_77_1","doi-asserted-by":"publisher","DOI":"10.1080\/10245330600938422"},{"key":"e_1_2_14_78_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70454-3"},{"key":"e_1_2_14_79_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2257.2000.00304.x"}],"container-title":["British Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fbjh.12950","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.12950","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T17:00:01Z","timestamp":1696352401000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bjh.12950"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,5,26]]},"references-count":78,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2014,7]]}},"alternative-id":["10.1111\/bjh.12950"],"URL":"https:\/\/doi.org\/10.1111\/bjh.12950","archive":["Portico"],"relation":{},"ISSN":["0007-1048","1365-2141"],"issn-type":[{"value":"0007-1048","type":"print"},{"value":"1365-2141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,5,26]]},"assertion":[{"value":"2014-01-30","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2014-03-31","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2014-05-26","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}