{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T15:06:14Z","timestamp":1779116774490,"version":"3.51.4"},"reference-count":24,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T00:00:00Z","timestamp":1764115200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T00:00:00Z","timestamp":1764115200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Transfusion"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Intrahepatic cholestasis, a severe form of sickle cell disease (SCD)\u2010related hepatopathy, is a rare complication of the disease, but with a continued high mortality rate. Although red cell exchange (RCE) is the first\u2010line intervention to manage individuals with sickle hepatopathy, RCE is often not enough to prevent or reverse multiorgan dysfunction (MODS), even in those with hemoglobin S (HbS) suppression below 30%.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Report<\/jats:title>\n                    <jats:p>An 18\u2010year\u2010old with sickle cell disease (HbSS genotype) presented with acute\u2010on\u2010chronic cholestasis secondary to sickle hepatopathy for liver transplant evaluation. However, due to improvement in his liver function after the implementation of a strict every four\u2010week RCE program, he was delisted and instead managed successfully for 5\u2009years on RCE. At age 24\u2009years, he again developed MODS with decompensated acute liver failure and anuric acute renal failure, despite RCE and HbS &lt;30%. He then underwent therapeutic plasma exchange (TPE) with 1:1 fresh frozen plasma\/albumin replacement (one plasma volume) with rapid improvement in both renal and synthetic liver function. He continued scheduled TPE as a bridge to successful deceased donor liver transplantation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Our case provides additional evidence of the efficacy of TPE after RCE as a temporizing measure to definitive therapy in the setting of MODS secondary to SCD\u2010related intrahepatic cholestasis. In addition, we show that liver transplantation for intrahepatic cholestasis, a disease with a historically high mortality rate, can be completed safely in patients with SCD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/trf.70011","type":"journal-article","created":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T12:39:04Z","timestamp":1764160744000},"page":"266-271","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Therapeutic plasma exchange as a bridge to successful liver transplantation in an adolescent with sickle hepatopathy"],"prefix":"10.1111","volume":"66","author":[{"given":"Samia Isabelle","family":"Perez","sequence":"first","affiliation":[{"name":"L\u00e9man Manhattan Preparatory School  New York City New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiang","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Cincinnati College of Medicine  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Benjamin H.","family":"Hinrichs","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Cincinnati College of Medicine  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0990-399X","authenticated-orcid":false,"given":"Kristine","family":"Karkoska","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Internal Medicine University of Cincinnati College of Medicine  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,11,26]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/aqab096"},{"issue":"5","key":"e_1_2_9_3_1","article-title":"Acute liver failure from sickle cell hepatopathy treated with exchange 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