{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T23:43:53Z","timestamp":1778715833835,"version":"3.51.4"},"reference-count":21,"publisher":"BMJ","issue":"1","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2020,11,26]]},"published-print":{"date-parts":[[2021,1]]},"abstract":"<jats:p>Prevalence of haemoglobin sickle-\u03b2<jats:sup>+<\/jats:sup> thalassaemia (Hb S\/\u03b2+thal) is variable with geography ranging from 0.2% to 10% among sickle cell patients. Clinical presentation of Hb S\/\u03b2+thal patients depends on HbA level, with milder disease often going undiagnosed. However, rarely these patients can present with a fulminant vaso-occlusive crisis (VOC). Given VOC can present with non-specific symptoms, the diagnosis and treatment is often delayed. Here, we present a patient who initially developed altered mental status, pancytopenia and multiorgan failure due a critical VOC resulting in bone marrow necrosis and fat embolism. Subsequent workup confirmed that our patient had Sickle-\u03b2<jats:sup>+<\/jats:sup> thalassaemia, which had gone undiagnosed, despite subclinical evidence of haemolysis on routine lab work for years. Following diagnosis and initiation of RBC exchange, he improved significantly and was discharged home. High index of suspicion and bone marrow biopsy is vital for early diagnosis and management of this rare condition.<\/jats:p>","DOI":"10.1136\/bcr-2020-238317","type":"journal-article","created":{"date-parts":[[2021,1,6]],"date-time":"2021-01-06T12:46:57Z","timestamp":1609937217000},"page":"e238317","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["Bone marrow necrosis and fat embolism syndrome: a near fatal complication in previously undiagnosed sickle beta + thalassaemia"],"prefix":"10.1136","volume":"14","author":[{"given":"Nibash","family":"Budhathoki","sequence":"first","affiliation":[{"name":"Hematology and Oncology, NYU Langone Hospital\u2014Long Island, Mineola, New York, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sunita","family":"Timilsina","sequence":"additional","affiliation":[{"name":"Geriatrics, NYU Langone Hospital\u2014Long Island, Mineola, New York, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bebu","family":"Ram","sequence":"additional","affiliation":[{"name":"Pathology, NYU Langone Hospital\u2014Long Island, Mineola, New York, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Douglas","family":"Marks","sequence":"additional","affiliation":[{"name":"Hematology and Oncology, NYU Langone Hospital\u2014Long Island, Mineola, New York, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2021,1,6]]},"reference":[{"key":"2025082707044779000_14.1.e238317.1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(10)61029-X"},{"key":"2025082707044779000_14.1.e238317.2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199709113371107"},{"key":"2025082707044779000_14.1.e238317.3","first-page":"206","article-title":"The hereditary haemolytic anaemias","volume":"8","author":"Dacie","year":"1974","journal-title":"J R Coll Physicians Lond"},{"key":"2025082707044779000_14.1.e238317.4","first-page":"4","article-title":"Differences in the clinical and genotypic presentation of sickle cell disease around the world","volume":"15","author":"Saraf","year":"2014","journal-title":"Paediatr Respir Rev"},{"key":"2025082707044779000_14.1.e238317.5","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/j.bjhh.2015.02.008","article-title":"The compound state: Hb S\/beta-thalassemia","volume":"37","author":"Figueiredo","year":"2015","journal-title":"Rev Bras Hematol Hemoter"},{"key":"2025082707044779000_14.1.e238317.6","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24711"},{"key":"2025082707044779000_14.1.e238317.7","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197301042880108"},{"key":"2025082707044779000_14.1.e238317.8","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1510865"},{"key":"2025082707044779000_14.1.e238317.9","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.13812"},{"key":"2025082707044779000_14.1.e238317.10","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21883"},{"key":"2025082707044779000_14.1.e238317.11","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1002\/ajh.23117","article-title":"Multiorgan failure and bone marrow necrosis in three adults with sickle cell-\u03b2+ -thalassemia","volume":"87","author":"Adamski","year":"2012","journal-title":"Am J Hematol"},{"key":"2025082707044779000_14.1.e238317.12","doi-asserted-by":"crossref","DOI":"10.1182\/blood.V124.21.4942.4942","article-title":"Catastrophic multi-organ failure with bone marrow necrosis in a sickle cell beta plus thalassemia patient","volume":"124","author":"McCollom","year":"2014","journal-title":"Blood"},{"key":"2025082707044779000_14.1.e238317.13","first-page":"2050313X1771314","article-title":"Pseudo-thrombotic thrombocytopenic purpura presenting as multi-organ dysfunction syndrome: a rare complication of pernicious anemia","volume":"5","author":"Kandel","year":"2017","journal-title":"SAGE Open Med Case Rep"},{"key":"2025082707044779000_14.1.e238317.14","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-05-498311"},{"key":"2025082707044779000_14.1.e238317.15","doi-asserted-by":"publisher","DOI":"10.1097\/00062752-200203000-00003"},{"key":"2025082707044779000_14.1.e238317.16","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2013.12.002"},{"key":"2025082707044779000_14.1.e238317.17","first-page":"670","article-title":"Bone marrow necrosis in sickle Cell-Beta thalassemia patient mimicking thrombotic thrombocytopenic purpura","volume":"48","author":"Myers","year":"2018","journal-title":"Ann Clin Lab Sci"},{"key":"2025082707044779000_14.1.e238317.18","doi-asserted-by":"crossref","first-page":"42","DOI":"10.4322\/acr.2017.043","article-title":"Bone marrow necrosis and fat embolism syndrome: a dreadful complication of hemoglobin sickle cell disease","volume":"7","author":"Targueta","year":"2017","journal-title":"Autops Case Rep"},{"key":"2025082707044779000_14.1.e238317.19","doi-asserted-by":"publisher","DOI":"10.1097\/00000441-200011000-00009"},{"key":"2025082707044779000_14.1.e238317.20","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20348"},{"key":"2025082707044779000_14.1.e238317.21","first-page":"875","article-title":"Survey of sickle cell disease in Italy","volume":"83","author":"Russo-Mancuso","year":"1998","journal-title":"Haematologica"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2020-238317","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T14:04:56Z","timestamp":1756303496000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2020-238317"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1]]},"references-count":21,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021,1,6]]},"published-print":{"date-parts":[[2021,1]]}},"alternative-id":["10.1136\/bcr-2020-238317"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2020-238317","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,1]]}}}