{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T20:38:43Z","timestamp":1791664723487,"version":"4.3.4"},"reference-count":17,"publisher":"Wiley","issue":"S1","license":[{"start":{"date-parts":[[2023,2,7]],"date-time":"2023-02-07T00:00:00Z","timestamp":1675728000000},"content-version":"vor","delay-in-days":6,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Transfusion"],"published-print":{"date-parts":[[2023,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Fat embolism syndrome (FES) is a rare complication, which was reported mostly with milder forms of heterozygous sickle cell disease (SCD). It may present in a catastrophic way with multi\u2010organ failure, particularly involving the pulmonary and neurological systems. Diagnosis is often missed or delayed; and the standard recommended treatment is red cell exchange (RCE) transfusion, which has sub\u2010optimal results, such as debilitating long\u2010term neurological complications. Recently, few reports suggested that the addition of Therapeutic Plasma Exchange (TPE) might further improve the outcome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case Description<\/jats:title><jats:p>A 23\u2010year\u2010old woman with homozygote SCD was admitted with bony pains and vaso\u2010occlusive crises. However, her course evolved to respiratory failure requiring mechanical ventilation, decreased level of consciousness, skin rash, severe anemia and thrombocytopenia and a picture consistent with thrombotic microangiopathy. MRI of the brain showed scattered multi\u2010focal ischemic foci and cytotoxic edema. The patient received RCE on the third day after admission without improvement. On the seventh day, TPE was instituted (2\u00a0L\/day of fresh frozen plasma for 5\u2009days), following which she regained her consciousness and showed an improvement in her laboratory abnormalities. On follow up, she had gradual full neurological recovery and resolution of the MRI findings within a few months.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>FES remains a diagnostic and therapeutic challenge, with significant morbidity and mortality. Success in the management of this reported case with the addition of TPE to RCE supports the notion that TPE may be a potentially helpful modality that deserves further research.<\/jats:p><\/jats:sec>","DOI":"10.1111\/trf.17220","type":"journal-article","created":{"date-parts":[[2023,2,7]],"date-time":"2023-02-07T10:38:16Z","timestamp":1675766296000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Successful therapeutic plasma exchange for a patient with sickle cell disease and fat embolism syndrome after a failure of a response to red cell exchange transfusion"],"prefix":"10.1111","volume":"63","author":[{"given":"Abdullah","family":"Alsaghir","sequence":"first","affiliation":[{"name":"Pulmonary &amp; Critical Care Medicine Dammam Medical Complex  Dammam Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lina","family":"Alsaghir","sequence":"additional","affiliation":[{"name":"Royal College of Surgeons in Ireland\u2013 Bahrain  Busaiteen Bahrain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janat","family":"Alsaif","sequence":"additional","affiliation":[{"name":"Imam Abdulrahman bin Faisal University  Dammam Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0880-206X","authenticated-orcid":false,"given":"Abdullah","family":"Mobeireek","sequence":"additional","affiliation":[{"name":"Department of Medicine King Faisal Specialist Hospital &amp; Research Centre  Riyadh Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,2,7]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm9113601"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.14423\/SMJ.0000000000000520"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.flm.2017.02.001"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1177\/23247096211012266"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20348"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2020-236743"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1080\/03630269.2021.1990081"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.15941"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.transci.2021.103226"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000002399"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ensci.2018.11.012"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41366-022-01071-w"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1155\/2021\/5519812"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.23384"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-6576.1997.tb04759.x"},{"issue":"5","key":"e_1_2_5_17_1","first-page":"670","article-title":"Bone marrow necrosis in sickle cell\u2010beta thalassemia patient mimicking thrombotic thrombocytopenic purpura","volume":"48","author":"Myers CF","year":"2018","journal-title":"Ann Clin Lab Sci"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-012-1647-3"}],"container-title":["Transfusion"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/trf.17220","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/trf.17220","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/trf.17220","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,19]],"date-time":"2023-08-19T05:11:36Z","timestamp":1692421896000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/trf.17220"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2]]},"references-count":17,"journal-issue":{"issue":"S1","published-print":{"date-parts":[[2023,2]]}},"alternative-id":["10.1111\/trf.17220"],"URL":"https:\/\/doi.org\/10.1111\/trf.17220","archive":["Portico"],"relation":{},"ISSN":["0041-1132","1537-2995"],"issn-type":[{"value":"0041-1132","type":"print"},{"value":"1537-2995","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,2]]},"assertion":[{"value":"2022-06-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-11-29","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-02-07","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}