{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T10:50:54Z","timestamp":1782384654855,"version":"3.54.5"},"reference-count":11,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T00:00:00Z","timestamp":1782345600000},"content-version":"vor","delay-in-days":25,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,5,31]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Hypersplenism in adults with homozygous sickle cell disease (HbSS) is rare, as most patients undergo autosplenectomy during early childhood. However, phenotypic variants such as high fetal hemoglobin, coexisting hemoglobinopathies, and different globin gene haplotypes can lead to splenic preservation and altered disease progression. These patients often exhibit milder symptoms, delayed complications, and increased morbidity. We report a rare case of a 43-year-old female with HbSS and elevated fetal hemoglobin who presented with hypersplenism, characterized by massive splenomegaly, pancytopenia, and compensatory bone marrow proliferation. She underwent an open splenectomy with an uneventful postoperative course. This case highlights the importance of recognizing atypical presentations in adult patients with sickle cell disease, in whom splenomegaly may persist. Clinicians should maintain a high index of suspicion to facilitate timely diagnosis and appropriate management, which can significantly improve patient outcomes.<\/jats:p>","DOI":"10.1093\/jscr\/rjag434","type":"journal-article","created":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T12:07:03Z","timestamp":1778760423000},"source":"Crossref","is-referenced-by-count":0,"title":["A rare presentation of hypersplenism in an adult female with homozygous sickle cell disease"],"prefix":"10.1093","volume":"2026","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6752-0092","authenticated-orcid":false,"given":"Parvat Kuwar","family":"Chhetri","sequence":"first","affiliation":[{"name":"Department of General Surgery, TMSS Medical college , Bogura ,","place":["Bangladesh"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3150-8937","authenticated-orcid":false,"given":"Pratiksha","family":"Paudel","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, All Nepal Hospital , Kathmandu ,","place":["Nepal"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-9606-0317","authenticated-orcid":false,"given":"Rahul","family":"Jha","sequence":"additional","affiliation":[{"name":"Department of General Surgery, National Academy of Medical Sciences , Kathmandu ,","place":["Nepal"]},{"name":"Department of General Surgery, Karnali Academy of Health Sciences , Jumla ,","place":["Nepal"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-1477-8958","authenticated-orcid":false,"given":"Rishika","family":"Prasad","sequence":"additional","affiliation":[{"name":"Department of General Surgery, Nepal Medical College Teaching Hospital , Kathmandu ,","place":["Nepal"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0371-4821","authenticated-orcid":false,"given":"Srikant","family":"Agrawal","sequence":"additional","affiliation":[{"name":"Department of General Surgery, National Academy of Medical Sciences , Kathmandu ,","place":["Nepal"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,6,3]]},"reference":[{"key":"2026062506252494700_ref1","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1111\/j.1365-2141.1991.tb08628.x","article-title":"Acute splenic sequestration in Jamaican adults with homozygous sickle cell disease: a role of alpha thalassaemia","volume":"77","author":"De Ceulaer","year":"1991","journal-title":"Br J Haematol"},{"key":"2026062506252494700_ref2","doi-asserted-by":"publisher","first-page":"198","DOI":"10.18502\/ijhoscr.v16i4.10877","article-title":"Prevalence of splenomegaly and splenic complications in adults with sickle cell disease and its relation to fetal hemoglobin","volume":"16","author":"Lakhani","year":"2022","journal-title":"Int J Hematol Oncol Stem Cell Res"},{"key":"2026062506252494700_ref3","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1182\/blood-2011-03-325258","article-title":"Fetal hemoglobin in sickle cell anemia","volume":"118","author":"Akinsheye","year":"2011","journal-title":"Blood"},{"key":"2026062506252494700_ref4","first-page":"1","volume-title":"Emery and Rimoin's Principles and Practice of Medical Genetics","author":"Old","year":"2013","edition":"6"},{"key":"2026062506252494700_ref5","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1179\/1607845412Y.0000000036","article-title":"Splenic enlargement in adults with homozygous sickle cell disease: the Jamaican experience","volume":"18","author":"Asnani","year":"2013","journal-title":"Hematology"},{"key":"2026062506252494700_ref6","doi-asserted-by":"publisher","first-page":"1233","DOI":"10.1002\/ajh.24872","article-title":"Fetal hemoglobin in sickle cell anemia: The Arab-Indian haplotype and new therapeutic agents","volume":"92","author":"Habara","year":"2017","journal-title":"Am J Hematol"},{"key":"2026062506252494700_ref7","doi-asserted-by":"publisher","first-page":"eaau6085","DOI":"10.1126\/sciimmunol.aau6085","article-title":"Structure and function of the immune system in the spleen","volume":"4","author":"Lewis","year":"2019","journal-title":"Sci Immunol"},{"key":"2026062506252494700_ref8","doi-asserted-by":"publisher","first-page":"2377","DOI":"10.3892\/etm.2016.3683","article-title":"Hypersplenism: history and current status","volume":"12","author":"Lv","year":"2016","journal-title":"Exp Ther Med"},{"key":"2026062506252494700_ref9","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1016\/j.amjsurg.2006.09.043","article-title":"Concomitant intraoperative splenic artery embolization and laparoscopic splenectomy versus laparoscopic splenectomy: comparison of treatment outcome","volume":"193","author":"Naoum","year":"2007","journal-title":"Am J Surg"},{"key":"2026062506252494700_ref10","doi-asserted-by":"publisher","first-page":"2758","DOI":"10.1007\/s00464-012-2270-z","article-title":"Comparative treatment and literature review for laparoscopic splenectomy alone versus preoperative splenic artery embolization splenectomy","volume":"26","author":"Wu","year":"2012","journal-title":"Surg Endosc"},{"key":"2026062506252494700_ref11","doi-asserted-by":"publisher","first-page":"961345","DOI":"10.1155\/2014\/961345","article-title":"Clinical application of partial splenic embolization","volume":"2014","author":"Guan","year":"2014","journal-title":"Sci World J."}],"container-title":["Journal of Surgical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jscr\/article-pdf\/2026\/6\/rjag434\/68610209\/rjag434.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jscr\/article-pdf\/2026\/6\/rjag434\/68610209\/rjag434.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T10:25:36Z","timestamp":1782383136000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jscr\/article\/doi\/10.1093\/jscr\/rjag434\/8700312"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,31]]},"references-count":11,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2026,5,31]]}},"URL":"https:\/\/doi.org\/10.1093\/jscr\/rjag434","relation":{},"ISSN":["2042-8812"],"issn-type":[{"value":"2042-8812","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,6]]},"published":{"date-parts":[[2026,5,31]]},"article-number":"rjag434"}}