{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T11:37:46Z","timestamp":1789904266984,"version":"4.0.1"},"reference-count":36,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2005,6,24]],"date-time":"2005-06-24T00:00:00Z","timestamp":1119571200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2005,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Sickle cell disease displays a unique progression in the Eastern province of Saudi Arabia, where splenomegaly with hypersplenism is noted with high frequency in the adolescent and adult patients. The late persistence of splenomegaly although likely reflects the milder progression of sickle cell disease in this region; nevertheless, it predisposes the patients to increased morbidity. The present study documents the characteristic clinicopathological features of splenomegaly associated with sickle cell disease in the Al\u2010Hassa region of Eastern province Saudi Arabia. Forty\u2010four cases of sickle cell disease patients in whom splenectomy was performed during 1999\u20132003 were studied. The hemoglobinopathy profiles of the patients (age range 5\u201342 years) comprised sickle cell anemia (8 cases), sickle cell anemia with high fetal hemoglobin (23 cases), and sickle cell\u2010\u03b2\u00b0 thalassemia (13 cases). All patients had manifestations of hypersplenism and 39 patients experienced episodes of minor\u2010type sequestration crisis. Splenectomy was effective in ameliorating the hematological abnormalities in all cases, without any major complications in the follow\u2010up period. The splenectomy specimens showed moderate\u2010to\u2010marked enlargement in most cases, with histological features of fibrocongestive splenomegaly and prominent Gandy\u2010gamma body formations. Micro\u2010infarcts in 27 cases and gross infarctions in 9 cases were evident. The relationship of persistent splenomegaly with higher fetal hemoglobin levels and splenic hypofunction is examined along with the significance of splenectomy in these cases. Am. J. Hematol. 79:180\u2013186, 2005. \u00a9 2005 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.20380","type":"journal-article","created":{"date-parts":[[2005,6,24]],"date-time":"2005-06-24T19:49:25Z","timestamp":1119642565000},"page":"180-186","source":"Crossref","is-referenced-by-count":28,"title":["Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia"],"prefix":"10.1002","volume":"79","author":[{"given":"Rajan","family":"Chopra","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdul Rahman","family":"Al\u2010Mulhim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed Taher","family":"Al\u2010Baharani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,6,24]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1991.tb08013.x"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(84)80950-6"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.1985.tb01570.x"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(89)80440-8"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1159\/000204654"},{"key":"e_1_2_1_7_2","first-page":"60","article-title":"Splenectomy for hematological disorders in the eastern province of Saudi Arabia","volume":"77","author":"Al\u2010Awamy","year":"1992","journal-title":"Int Surg"},{"key":"e_1_2_1_8_2","first-page":"251","article-title":"Relationship of hemoglobin F and \u03b1\u2010thalassemia to severity of sickle cell anemia in the eastern province of Saudi Arabia","volume":"6","author":"Al\u2010Awamy BH","year":"1968","journal-title":"Ann Trop Pediatr"},{"issue":"6","key":"e_1_2_1_9_2","first-page":"1154","article-title":"Haplotypes of the b\u2010globin gene as prognostic factors in sickle cell disease","volume":"5","author":"El\u2010Hazmi MAF","year":"1999","journal-title":"East Medit Health Jl"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(97)07330-3"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1148\/radiographics.21.4.g01jl23971"},{"key":"e_1_2_1_12_2","first-page":"1263","volume-title":"Wintrobe's clinical hematology","author":"Wang WC","year":"2004"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9610(96)00158-4"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00174580"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1080\/02724936.1989.11748610"},{"issue":"4","key":"e_1_2_1_16_2","first-page":"343","article-title":"The spleen in sickle cell disease and thalassemia","volume":"1","author":"Powars DR","year":"1979","journal-title":"Am J Pediatr Hematol Oncol"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.10049"},{"issue":"3","key":"e_1_2_1_18_2","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1080\/02724936.1985.11748380","article-title":"Hypersplenism in homozygous sickle\u2010cell disease","volume":"5","author":"Mallouh AA","year":"1985","journal-title":"Ann Trop Paediatr"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70454-3"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(200002)63:2<68::AID-AJH2>3.0.CO;2-N"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM196910232811703"},{"issue":"3","key":"e_1_2_1_22_2","doi-asserted-by":"crossref","first-page":"217","DOI":"10.5144\/0256-4947.1998.217","article-title":"Sonographic assessment of spleen size in Saudi patients with sickle cell disease","volume":"18","author":"Al\u2010Salem AH","year":"1998","journal-title":"Ann Saudi Med"},{"key":"e_1_2_1_23_2","first-page":"434","article-title":"Splenic and hepatic function in sickle cell anemia, with emphasis on patients from the Eastern Province of Saudi Arabia","volume":"7","author":"Al\u2010Awamy BH","year":"1986","journal-title":"Saudi Med J"},{"key":"e_1_2_1_24_2","first-page":"231","volume-title":"Dacie and Lewis practical haematology","author":"Wild BJ","year":"2001"},{"issue":"6","key":"e_1_2_1_25_2","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.26719\/1999.5.6.1147","article-title":"Appraisal of sickle cell and thalassemia genes in Saudi Arabia","volume":"5","author":"El\u2010Hazmi MAF","year":"1999","journal-title":"East Medit Health J"},{"key":"e_1_2_1_26_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00926521"},{"key":"e_1_2_1_27_2","doi-asserted-by":"publisher","DOI":"10.1093\/tropej\/39.1.23"},{"key":"e_1_2_1_28_2","doi-asserted-by":"publisher","DOI":"10.1159\/000153927"},{"key":"e_1_2_1_29_2","doi-asserted-by":"publisher","DOI":"10.1159\/000206194"},{"key":"e_1_2_1_30_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)80529-3"},{"issue":"4","key":"e_1_2_1_31_2","doi-asserted-by":"crossref","first-page":"325","DOI":"10.5144\/0256-4947.1999.325","article-title":"Splenectomy for hematological diseases: the Qatif Central Hospital experience","volume":"19","author":"Al\u2010Salem AH","year":"1999","journal-title":"Ann Saudi Med"},{"issue":"1","key":"e_1_2_1_32_2","first-page":"33","article-title":"Indications for splenectomy among children with sickle cell disease in Kuwait","volume":"34","author":"Adekile A","year":"2002","journal-title":"Kuwait Med J"},{"key":"e_1_2_1_33_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(86)92205-1"},{"key":"e_1_2_1_34_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1991.tb08589.x"},{"key":"e_1_2_1_35_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(90)90419-6"},{"key":"e_1_2_1_36_2","doi-asserted-by":"crossref","first-page":"1742","DOI":"10.1182\/blood.V69.6.1742.1742","article-title":"Fetal hemoglobin levels and beta s globin haplotypes in an Indian population with sickle cell disease","volume":"69","author":"Kulozik AE","year":"1987","journal-title":"Blood"},{"key":"e_1_2_1_37_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1986.tb04117.x"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.20380","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.20380","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,16]],"date-time":"2023-11-16T22:25:34Z","timestamp":1700173534000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.20380"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,6,24]]},"references-count":36,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2005,7]]}},"alternative-id":["10.1002\/ajh.20380"],"URL":"https:\/\/doi.org\/10.1002\/ajh.20380","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,6,24]]}}}