{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T23:18:19Z","timestamp":1787008699041,"version":"build-2736575974"},"reference-count":11,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2020,5,4]],"date-time":"2020-05-04T00:00:00Z","timestamp":1588550400000},"content-version":"vor","delay-in-days":1,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Swedish Heart and Lung Foundation","award":["20160671"],"award-info":[{"award-number":["20160671"]}]},{"name":"ALF\/LUA","award":["ALFGBG-21431"],"award-info":[{"award-number":["ALFGBG-21431"]}]},{"name":"Sahlgrenska Academy and University Hospital"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,6,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Haemophagocytic lymphohistiocytosis (HLH) is an uncommon but serious systemic inflammatory response with high mortality rates. It can be triggered by malignancy or infectious agents, often in the context of immunosuppression. Literature covering HLH in heart transplantation (HTx) is scarce.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case summary<\/jats:title>\n                    <jats:p>A 25-year-old male with a history of celiac disease underwent HTx at Sahlgrenska Hospital in 2011 due to giant cell myocarditis and was treated with tacrolimus, mycophenolate mofetil (MMF), and prednisolone. He developed several episodes of acute cellular rejections (ACR) during the first 3 post-HTx years, which subsided after addition of everolimus. In May 2017, the patient was admitted to the hospital due to fever without focal symptoms. He had an extensive inflammatory reaction, but screening for infectious agents was negative. Haemophagocytic lymphohistiocytosis was discussed early, but first dismissed since two bone marrow biopsies revealed no signs of haemophagocytosis. Increasing levels of soluble IL-2 were considered confirmative of the diagnosis. Even with intense immunosuppressant treatment, the patient deteriorated and died in progressive multiorgan failure within 2\u2009weeks of the symptom onset.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>A 25-year-old HTx recipient with an extensive inflammatory response, fulfilled criteria for HLH, but the diagnosis was delayed due to normal bone marrow biopsies. A background with autoimmune reactivity and immunosuppressive therapy may have contributed to HLH, but the actual trigger was not identified. Haemophagocytic lymphohistiocytosis can occur in HTx recipients in the absence of malignancy, identifiable infectious triggers and signs of haemophagocytosis. Early diagnosis and intervention are likely to be of importance for a favourable outcome.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytaa070","type":"journal-article","created":{"date-parts":[[2020,5,3]],"date-time":"2020-05-03T16:48:23Z","timestamp":1588524503000},"page":"1-4","source":"Crossref","is-referenced-by-count":4,"title":["Haemophagocytic lymphohistiocytosis after heart transplantation: a case report"],"prefix":"10.1093","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3184-1833","authenticated-orcid":false,"given":"Christian","family":"Danielsson","sequence":"first","affiliation":[{"name":"Department of Transplant Institute, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2802-1191","authenticated-orcid":false,"given":"Kristjan","family":"Karason","sequence":"first","affiliation":[{"name":"Department of Transplant Institute, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden"},{"name":"Department of Cardiology, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4961-9704","authenticated-orcid":false,"given":"G\u00f6ran","family":"Dellgren","sequence":"first","affiliation":[{"name":"Department of Transplant Institute, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden"},{"name":"Department of Cardiothoracic Surgery, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden"},{"name":"Department of Molecular and Clinical Medicine, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, SE-413 90 Gothenburg, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,5,3]]},"reference":[{"key":"2020062620243420700_ytaa070-B1","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1016\/S1053-2498(02)00817-3","article-title":"Reactive hemophagocytic syndrome associated with disseminated histoplasmosis in a heart transplant recipient","volume":"22","author":"Masri","year":"2003","journal-title":"J Heart Lung Transplant"},{"key":"2020062620243420700_ytaa070-B2","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1182\/asheducation-2005.1.82","article-title":"Familial and acquired hemophagocytic lymphohistiocytosis","volume":"2005","author":"Janka","year":"2005","journal-title":"Hematol Am Soc Hematol Educ Program"},{"key":"2020062620243420700_ytaa070-B3","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1182\/asheducation-2009.1.127","article-title":"Hemophagocytic lymphohistiocytosis (HLH) and related disorders","volume":"2009","author":"Filipovich","year":"2009","journal-title":"Hematol Am Soc Hematol Educ Program"},{"key":"2020062620243420700_ytaa070-B4","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/S1054-8807(97)00060-4","article-title":"Hemophagocytic syndrome complicating cardiac transplantation","volume":"7","author":"Burns","year":"1998","journal-title":"Cardiovasc Pathol"},{"key":"2020062620243420700_ytaa070-B5","doi-asserted-by":"crossref","first-page":"1845","DOI":"10.3109\/10428194.2012.666664","article-title":"Aggressive plasmablastic lymphoproliferation complicated by hemophagocytic syndrome 12 years after heart transplant","volume":"53","author":"Reuland","year":"2012","journal-title":"Leuk Lymphoma"},{"key":"2020062620243420700_ytaa070-B6","doi-asserted-by":"crossref","first-page":"3121","DOI":"10.1128\/JCM.00758-14","article-title":"Disseminated Mycobacterium lentiflavum responsible for hemophagocytic lymphohistocytosis in a man with a history of heart transplantation","volume":"52","author":"Thomas","year":"2014","journal-title":"J Clin Microbiol"},{"key":"2020062620243420700_ytaa070-B7","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1002\/pbc.21039","article-title":"HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis","volume":"48","author":"Henter","year":"2007","journal-title":"Pediatr Blood Cancer"},{"key":"2020062620243420700_ytaa070-B8","first-page":"230","article-title":"Myocardial involvement due to a disseminated human cytomegalovirus infection in a heart transplant recipient. 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