{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T10:30:21Z","timestamp":1788517821807,"version":"build-2803163510"},"reference-count":87,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2023,12,25]],"date-time":"2023-12-25T00:00:00Z","timestamp":1703462400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2023,12,25]],"date-time":"2023-12-25T00:00:00Z","timestamp":1703462400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Adv Ther"],"published-print":{"date-parts":[[2024,2]]},"DOI":"10.1007\/s12325-023-02768-8","type":"journal-article","created":{"date-parts":[[2023,12,25]],"date-time":"2023-12-25T12:01:52Z","timestamp":1703505712000},"page":"553-566","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Liver Dysfunction in Adult Hemophagocytic Lymphohistiocytosis: A Narrative Review"],"prefix":"10.1007","volume":"41","author":[{"given":"Muaaz","family":"Masood","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Asma","family":"Siddique","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajesh","family":"Krishnamoorthi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5219-6277","authenticated-orcid":false,"given":"Richard A.","family":"Kozarek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,12,25]]},"reference":[{"issue":"15","key":"2768_CR1","doi-asserted-by":"publisher","first-page":"4041","DOI":"10.1182\/blood-2011-03-278127","volume":"118","author":"MB Jordan","year":"2011","unstructured":"Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041\u201352.","journal-title":"Blood"},{"issue":"4","key":"2768_CR2","doi-asserted-by":"publisher","first-page":"486","DOI":"10.3324\/haematol.10829","volume":"92","author":"AJ Ferreri","year":"2007","unstructured":"Ferreri AJ, Dognini GP, Campo E, et al. Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions. Haematologica. 2007;92(4):486\u201392.","journal-title":"Haematologica"},{"issue":"11","key":"2768_CR3","doi-asserted-by":"publisher","DOI":"10.1097\/HS9.0000000000000797","volume":"6","author":"J West","year":"2022","unstructured":"West J, Stilwell P, Liu H, et al. Temporal trends in the incidence of hemophagocytic lymphohistiocytosis: a nationwide cohort study from England 2003\u20132018. Hemasphere. 2022;6(11):e797.","journal-title":"Hemasphere"},{"key":"2768_CR4","doi-asserted-by":"crossref","unstructured":"Abdelhay A, Mahmoud AA, Al Ali O, Hashem A, Orakzai A, Jamshed S. Epidemiology, characteristics, and outcomes of adult haemophagocytic lymphohistiocytosis in the USA, 2006\u201319: a national, retrospective cohort study. eClinicalMedicine. 2023;62:102143.","DOI":"10.1016\/j.eclinm.2023.102143"},{"issue":"2","key":"2768_CR5","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1002\/pbc.21039","volume":"48","author":"JI Henter","year":"2007","unstructured":"Henter JI, Horne A, Aric\u00f3 M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124\u201331.","journal-title":"Pediatr Blood Cancer"},{"issue":"3","key":"2768_CR6","doi-asserted-by":"publisher","first-page":"220","DOI":"10.1002\/ajh.23911","volume":"90","author":"ZK Otrock","year":"2015","unstructured":"Otrock ZK, Eby CS. Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol. 2015;90(3):220\u20134.","journal-title":"Am J Hematol"},{"issue":"2","key":"2768_CR7","doi-asserted-by":"publisher","first-page":"100","DOI":"10.1097\/MD.0000000000000022","volume":"93","author":"J Li","year":"2014","unstructured":"Li J, Wang Q, Zheng W, et al. Hemophagocytic lymphohistiocytosis: clinical analysis of 103 adult patients. Medicine (Baltimore). 2014;93(2):100\u20135.","journal-title":"Medicine (Baltimore)"},{"issue":"8","key":"2768_CR8","doi-asserted-by":"publisher","first-page":"3205","DOI":"10.1007\/s10620-023-07985-3","volume":"68","author":"M Masood","year":"2023","unstructured":"Masood M, Siddique A, Kozarek RA. Adult hemophagocytic lymphohistiocytosis mimicking acute viral hepatitis. Dig Dis Sci. 2023;68(8):3205\u20137.","journal-title":"Dig Dis Sci"},{"issue":"6047","key":"2768_CR9","doi-asserted-by":"publisher","first-page":"194","DOI":"10.1016\/S0140-6736(00)61951-7","volume":"234","author":"R Bodley Scott","year":"1939","unstructured":"Bodley Scott R, Robb-Smith AHT. Histiocytic medullary reticulosis. Lancet. 1939;234(6047):194\u20138.","journal-title":"Lancet"},{"issue":"136","key":"2768_CR10","doi-asserted-by":"publisher","first-page":"519","DOI":"10.1136\/adc.27.136.519","volume":"27","author":"JW Farquhar","year":"1952","unstructured":"Farquhar JW, Claireaux AE. Familial haemophagocytic reticulosis. Arch Dis Child. 1952;27(136):519\u201325.","journal-title":"Arch Dis Child"},{"key":"2768_CR11","doi-asserted-by":"crossref","unstructured":"Janka G, Imashuku S, Elinder G, Schneider M, Henter JI. Infection- and malignancy-associated hemophagocytic syndromes. Secondary hemophagocytic lymphohistiocytosis. Hematol Oncol Clin North Am. 1998;12(2):435\u201344.","DOI":"10.1016\/S0889-8588(05)70521-9"},{"issue":"12","key":"2768_CR12","doi-asserted-by":"publisher","first-page":"2754","DOI":"10.1182\/blood-2011-08-374199","volume":"119","author":"YT Bryceson","year":"2012","unstructured":"Bryceson YT, Pende D, Maul-Pavicic A, et al. A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes. Blood. 2012;119(12):2754\u201363.","journal-title":"Blood"},{"issue":"3","key":"2768_CR13","doi-asserted-by":"publisher","first-page":"424","DOI":"10.1002\/pbc.22359","volume":"54","author":"JA Niece","year":"2010","unstructured":"Niece JA, Rogers ZR, Ahmad N, Langevin AM, McClain KL. Hemophagocytic lymphohistiocytosis in Texas: observations on ethnicity and race. Pediatr Blood Cancer. 2010;54(3):424\u20138.","journal-title":"Pediatr Blood Cancer"},{"key":"2768_CR14","doi-asserted-by":"publisher","first-page":"117","DOI":"10.2147\/OARRR.S151013","volume":"10","author":"B Lerkvaleekul","year":"2018","unstructured":"Lerkvaleekul B, Vilaiyuk S. Macrophage activation syndrome: early diagnosis is key. Open Access Rheumatol. 2018;10:117\u201328.","journal-title":"Open Access Rheumatol"},{"issue":"8","key":"2768_CR15","doi-asserted-by":"publisher","first-page":"1223","DOI":"10.1007\/s10067-012-1998-0","volume":"31","author":"CI Lin","year":"2012","unstructured":"Lin CI, Yu HH, Lee JH, et al. Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases. Clin Rheumatol. 2012;31(8):1223\u201330.","journal-title":"Clin Rheumatol"},{"issue":"6","key":"2768_CR16","doi-asserted-by":"publisher","first-page":"601","DOI":"10.3201\/eid0606.000608","volume":"6","author":"DN Fisman","year":"2000","unstructured":"Fisman DN. Hemophagocytic syndromes and infection. Emerg Infect Dis. 2000;6(6):601\u20138.","journal-title":"Emerg Infect Dis"},{"key":"2768_CR17","doi-asserted-by":"publisher","first-page":"1102","DOI":"10.3389\/fimmu.2017.01102","volume":"8","author":"E Brisse","year":"2017","unstructured":"Brisse E, Wouters CH, Andrei G, Matthys P. How viruses contribute to the pathogenesis of hemophagocytic lymphohistiocytosis. Front Immunol. 2017;8:1102.","journal-title":"Front Immunol"},{"issue":"35","key":"2768_CR18","doi-asserted-by":"publisher","first-page":"59977","DOI":"10.18632\/oncotarget.19230","volume":"8","author":"H Wang","year":"2017","unstructured":"Wang H, Xiong L, Tang W, Zhou Y, Li F. A systematic review of malignancy-associated hemophagocytic lymphohistiocytosis that needs more attentions. Oncotarget. 2017;8(35):59977\u201385.","journal-title":"Oncotarget"},{"issue":"6","key":"2768_CR19","doi-asserted-by":"publisher","first-page":"405","DOI":"10.1097\/ACI.0000000000000405","volume":"17","author":"CA Wysocki","year":"2017","unstructured":"Wysocki CA. Comparing hemophagocytic lymphohistiocytosis in pediatric and adult patients. Curr Opin Allergy Clin Immunol. 2017;17(6):405\u201313.","journal-title":"Curr Opin Allergy Clin Immunol"},{"issue":"4","key":"2768_CR20","doi-asserted-by":"publisher","first-page":"663","DOI":"10.1007\/s00296-016-3601-5","volume":"37","author":"L Garc\u00eda-Montoya","year":"2017","unstructured":"Garc\u00eda-Montoya L, S\u00e1enz-Tenorio CN, Janta I, et al. Hemophagocytic lymphohistiocytosis in a patient with Sj\u00f6gren\u2019s syndrome: case report and review. Rheumatol Int. 2017;37(4):663\u20139.","journal-title":"Rheumatol Int"},{"issue":"12","key":"2768_CR21","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0226043","volume":"14","author":"G Prencipe","year":"2019","unstructured":"Prencipe G, Bracaglia C, Caiello I, et al. The interferon-gamma pathway is selectively up-regulated in the liver of patients with secondary hemophagocytic lymphohistiocytosis. PLoS ONE. 2019;14(12):e0226043.","journal-title":"PLoS ONE"},{"issue":"4","key":"2768_CR22","doi-asserted-by":"publisher","first-page":"470","DOI":"10.5114\/ceji.2015.56971","volume":"40","author":"K Popko","year":"2015","unstructured":"Popko K, G\u00f3rska E. The role of natural killer cells in pathogenesis of autoimmune diseases. Cent Eur J Immunol. 2015;40(4):470\u20136.","journal-title":"Cent Eur J Immunol"},{"key":"2768_CR23","doi-asserted-by":"publisher","first-page":"335","DOI":"10.3389\/fimmu.2012.00335","volume":"3","author":"K Krzewski","year":"2012","unstructured":"Krzewski K, Coligan JE. Human NK cell lytic granules and regulation of their exocytosis. Front Immunol. 2012;3:335.","journal-title":"Front Immunol"},{"issue":"S1","key":"2768_CR24","doi-asserted-by":"publisher","first-page":"S17","DOI":"10.5045\/br.2021.2020323","volume":"56","author":"YR Kim","year":"2021","unstructured":"Kim YR, Kim DY. Current status of the diagnosis and treatment of hemophagocytic lymphohistiocytosis in adults. Blood Res. 2021;56(S1):S17\u2013S25.","journal-title":"Blood Res"},{"issue":"9927","key":"2768_CR25","doi-asserted-by":"publisher","first-page":"1503","DOI":"10.1016\/S0140-6736(13)61048-X","volume":"383","author":"M Ramos-Casals","year":"2014","unstructured":"Ramos-Casals M, Brito-Zer\u00f3n P, L\u00f3pez-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet. 2014;383(9927):1503\u201316.","journal-title":"Lancet"},{"key":"2768_CR26","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1186\/s13023-015-0224-y","volume":"10","author":"F Li","year":"2015","unstructured":"Li F, Yang Y, Jin F, et al. Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China. Orphanet J Rare Dis. 2015;10:20.","journal-title":"Orphanet J Rare Dis"},{"issue":"5","key":"2768_CR27","first-page":"1356","volume":"17","author":"LZ Yang","year":"2009","unstructured":"Yang LZ, Wang JS, Wang Z. Preliminary clinical analysis of the hepatic dysfunction in patients with acquired hemophagocytic lymphohistiocytosis. Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2009;17(5):1356\u20139.","journal-title":"Zhongguo Shi Yan Xue Ye Xue Za Zhi"},{"issue":"7","key":"2768_CR28","first-page":"507","volume":"104","author":"CM Tai","year":"2005","unstructured":"Tai CM, Liu CJ, Yao M. Successful treatment of acute hepatitis A-associated hemophagocytic syndrome by intravenous immunoglobulin. J Formos Med Assoc. 2005;104(7):507\u201310.","journal-title":"J Formos Med Assoc"},{"issue":"2","key":"2768_CR29","doi-asserted-by":"publisher","first-page":"232","DOI":"10.29271\/jcpsp.2021.02.232","volume":"31","author":"A Dogan","year":"2021","unstructured":"Dogan A, Demircioglu S, Ekinci O. Acute hepatitis-a virus infection as a rare cause of hemophagocytic lymphohistiocytosis. J Coll Physicians Surg Pak. 2021;31(2):232\u20134.","journal-title":"J Coll Physicians Surg Pak"},{"issue":"2","key":"2768_CR30","doi-asserted-by":"publisher","DOI":"10.14309\/crj.0000000000000532","volume":"8","author":"H Blaney","year":"2021","unstructured":"Blaney H, Thotakura D, Sisco L. Hemophagocytic lymphohistiocytosis associated with hepatitis B and HIV coinfection with resultant liver failure. ACG Case Rep J. 2021;8(2):e00532.","journal-title":"ACG Case Rep J"},{"issue":"8","key":"2768_CR31","doi-asserted-by":"publisher","DOI":"10.1002\/ccr3.4328","volume":"9","author":"M Bendari","year":"2021","unstructured":"Bendari M, Delsa H, Bouanani N, et al. Hemophagocytic lymphohistiocytosis associated with HBV-HCV coinfection in adult: Case report. Clin Case Rep. 2021;9(8):e04328.","journal-title":"Clin Case Rep"},{"key":"2768_CR32","doi-asserted-by":"publisher","DOI":"10.1016\/j.idcr.2022.e01465","volume":"28","author":"DT Chen","year":"2022","unstructured":"Chen DT, Patel I, Cabral S, Chintalapati S, Iddings A, Patel D. Hemophagocytic lymphohistiocytosis presenting in a patient with human immunodeficiency virus and reactivated hepatitis B infection. IDCases. 2022;28:e01465.","journal-title":"IDCases"},{"issue":"2","key":"2768_CR33","doi-asserted-by":"publisher","first-page":"103","DOI":"10.2169\/internalmedicine.53.1013","volume":"53","author":"M Saito","year":"2014","unstructured":"Saito M, Yano Y, Minami A, et al. Autoimmune-associated hemophagocytic syndrome originating from autoimmune hepatitis with a successful response to therapy. Intern Med. 2014;53(2):103\u20137.","journal-title":"Intern Med"},{"issue":"3","key":"2768_CR34","volume":"15","author":"SA Khan","year":"2023","unstructured":"Khan SA, Amir M. Hemophagocytic lymphohistiocytosis masquerading as autoimmune hepatitis. Cureus. 2023;15(3):e36543.","journal-title":"Cureus"},{"key":"2768_CR35","first-page":"3580796","volume":"2019","author":"C Casault","year":"2019","unstructured":"Casault C, Posadas-Calleja JG. Secondary hemophagocytic lymphohistiocytosis: a challenging diagnosis in a patient with autoimmune hepatitis. Case Rep Crit Care. 2019;2019:3580796.","journal-title":"Case Rep Crit Care"},{"issue":"3","key":"2768_CR36","doi-asserted-by":"publisher","first-page":"e49","DOI":"10.1016\/j.clinre.2017.10.007","volume":"42","author":"H Yildiz","year":"2018","unstructured":"Yildiz H, Vandercam B, Thissen X, et al. Hepatitis during pregnancy: a case of hemophagocytic lymphohistiocytosis. Clin Res Hepatol Gastroenterol. 2018;42(3):e49\u201355.","journal-title":"Clin Res Hepatol Gastroenterol"},{"key":"2768_CR37","doi-asserted-by":"publisher","DOI":"10.1016\/j.idcr.2018.e00413","volume":"14","author":"F Lutfi","year":"2018","unstructured":"Lutfi F, Patel A, Becker D, Shahid M, Shah K. Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failure. IDCases. 2018;14:e00413.","journal-title":"IDCases"},{"issue":"47","key":"2768_CR38","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000005431","volume":"95","author":"S Lin","year":"2016","unstructured":"Lin S, Li Y, Long J, Liu Q, Yang F, He Y. Acute liver failure caused by hemophagocytic lymphohistiocytosis in adults: a case report and review of the literature. Medicine (Baltimore). 2016;95(47):e5431.","journal-title":"Medicine (Baltimore)"},{"issue":"3","key":"2768_CR39","doi-asserted-by":"publisher","first-page":"184","DOI":"10.1016\/j.jceh.2017.01.119","volume":"7","author":"N Jagtap","year":"2017","unstructured":"Jagtap N, Sharma M, Rajesh G, et al. Hemophagocytic lymphohistiocytosis masquerading as acute liver failure: a single center experience. J Clin Exp Hepatol. 2017;7(3):184\u20139.","journal-title":"J Clin Exp Hepatol"},{"issue":"9","key":"2768_CR40","doi-asserted-by":"publisher","first-page":"629","DOI":"10.4254\/wjh.v10.i9.629","volume":"10","author":"MS Cappell","year":"2018","unstructured":"Cappell MS, Hader I, Amin M. Acute liver failure secondary to severe systemic disease from fatal hemophagocytic lymphohistiocytosis: case report and systematic literature review. World J Hepatol. 2018;10(9):629\u201336.","journal-title":"World J Hepatol"},{"issue":"9","key":"2768_CR41","volume":"12","author":"JJ Chango Azanza","year":"2020","unstructured":"Chango Azanza JJ, Lopetegui Lia N, Calle Sarmiento PM. Bile cast nephropathy secondary to hemophagocytic lymphohistiocytosis with liver failure. Cureus. 2020;12(9):e10226.","journal-title":"Cureus"},{"issue":"4","key":"2768_CR42","doi-asserted-by":"publisher","DOI":"10.14309\/crj.2016.135","volume":"3","author":"JM Giard","year":"2016","unstructured":"Giard JM, Decker KA, Lai JC, Gill RM, Logan AC, Fix OK. Acute liver failure secondary to hemophagocytic lymphohistiocytosis during pregnancy. ACG Case Rep J. 2016;3(4):e162.","journal-title":"ACG Case Rep J"},{"issue":"3","key":"2768_CR43","volume":"14","author":"M Qureshi","year":"2022","unstructured":"Qureshi M, Alabd A, Behling E, Schwarting R, Haroldson K. Acute liver failure in hemophagocytic lymphohistiocytosis secondary to metastatic renal cell carcinoma: a diagnostic dilemma. Cureus. 2022;14(3):e23455.","journal-title":"Cureus"},{"key":"2768_CR44","doi-asserted-by":"publisher","unstructured":"Pinto-Patarroyo GP, Rytting ME, Vierling JM, Suarez-Almazor ME. Haemophagocytic lymphohistiocytosis presenting as liver failure following Epstein-Barr and prior hepatitis A infections. BMJ Case Rep. 2013;2013:bcr2013008979. https:\/\/doi.org\/10.1136\/bcr-2013-008979.","DOI":"10.1136\/bcr-2013-008979"},{"key":"2768_CR45","doi-asserted-by":"publisher","DOI":"10.14309\/crj.2017.68","volume":"4","author":"R Patel","year":"2017","unstructured":"Patel R, Patel H, Mulvoy W, Kapoor S. Diffuse large B-Cell lymphoma with secondary hemophagocytic lymphohistiocytosis presenting as acute liver failure. ACG Case Rep J. 2017;4:e68.","journal-title":"ACG Case Rep J"},{"key":"2768_CR46","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.825806","volume":"13","author":"K Nakajima","year":"2022","unstructured":"Nakajima K, Hiejima E, Nihira H, et al. Case report: a case of Epstein-Barr virus-associated acute liver failure requiring hematopoietic cell transplantation after emergent liver transplantation. Front Immunol. 2022;13:825806.","journal-title":"Front Immunol"},{"issue":"1","key":"2768_CR47","doi-asserted-by":"publisher","first-page":"99","DOI":"10.1055\/s-0036-1571299","volume":"36","author":"A Schneier","year":"2016","unstructured":"Schneier A, Stueck AE, Petersen B, Thung SN, Perumalswami P. An unusual cause of acute liver failure: three cases of hemophagocytic lymphohistiocytosis presenting at a transplant center. Semin Liver Dis. 2016;36(1):99\u2013105.","journal-title":"Semin Liver Dis"},{"issue":"1","key":"2768_CR48","doi-asserted-by":"publisher","first-page":"8125","DOI":"10.1038\/s41598-019-43909-w","volume":"9","author":"J Dong","year":"2019","unstructured":"Dong J, Xie F, Jia L, et al. Clinical characteristics of liver failure with hemophagocytic lymphohistiocytosis. Sci Rep. 2019;9(1):8125.","journal-title":"Sci Rep"},{"key":"2768_CR49","doi-asserted-by":"publisher","unstructured":"Padhi S, Sarangi R, Patra S, Chandra Samal S. Hepatic involvement in hemophagocytic lymphohistiocytosis. 2019. In: Streba CT, Vere CC, Rogoveanu I, Tripodi V, Lucangioli S, editors. Hepatitis A and other associated hepatobiliary diseases. https:\/\/doi.org\/10.5772\/intechopen.90238IntechOpen; 2020.","DOI":"10.5772\/intechopen.90238"},{"issue":"6","key":"2768_CR50","doi-asserted-by":"publisher","first-page":"363","DOI":"10.1111\/j.1600-0676.1992.tb00590.x","volume":"12","author":"WM Tsui","year":"1992","unstructured":"Tsui WM, Wong KF, Tse CC. Liver changes in reactive haemophagocytic syndrome. Liver. 1992;12(6):363\u20137.","journal-title":"Liver"},{"issue":"4","key":"2768_CR51","doi-asserted-by":"publisher","first-page":"310","DOI":"10.1046\/j.1365-2559.1998.00377.x","volume":"32","author":"A Ost","year":"1998","unstructured":"Ost A, Nilsson-Ardnor S, Henter JI. Autopsy findings in 27 children with haemophagocytic lymphohistiocytosis. Histopathology. 1998;32(4):310\u20136.","journal-title":"Histopathology"},{"issue":"10","key":"2768_CR52","doi-asserted-by":"publisher","first-page":"775","DOI":"10.2169\/internalmedicine.48.1677","volume":"48","author":"M Okamoto","year":"2009","unstructured":"Okamoto M, Yamaguchi H, Isobe Y, et al. Analysis of triglyceride value in the diagnosis and treatment response of secondary hemophagocytic syndrome. Intern Med. 2009;48(10):775\u201381.","journal-title":"Intern Med"},{"issue":"3","key":"2768_CR53","doi-asserted-by":"publisher","first-page":"306","DOI":"10.1086\/345903","volume":"36","author":"DL Palazzi","year":"2003","unstructured":"Palazzi DL, McClain KL, Kaplan SL. Hemophagocytic syndrome in children: an important diagnostic consideration in fever of unknown origin. Clin Infect Dis. 2003;36(3):306\u201312.","journal-title":"Clin Infect Dis"},{"issue":"9","key":"2768_CR54","doi-asserted-by":"publisher","first-page":"1245","DOI":"10.1002\/lt.24485","volume":"22","author":"AZ Amir","year":"2016","unstructured":"Amir AZ, Ling SC, Naqvi A, et al. Liver transplantation for children with acute liver failure associated with secondary hemophagocytic lymphohistiocytosis. Liver Transpl. 2016;22(9):1245\u201353.","journal-title":"Liver Transpl"},{"issue":"5","key":"2768_CR55","doi-asserted-by":"publisher","first-page":"508","DOI":"10.6002\/ect.2011.0204","volume":"10","author":"G Wright","year":"2012","unstructured":"Wright G, Wilmore S, Makanyanga J, et al. Liver transplant for adult hemophagocytic lymphohistiocytosis: case report and literature review. Exp Clin Transplant. 2012;10(5):508\u201312.","journal-title":"Exp Clin Transplant"},{"issue":"8","key":"2768_CR56","doi-asserted-by":"publisher","DOI":"10.1111\/ctr.14392","volume":"35","author":"J Chesner","year":"2021","unstructured":"Chesner J, Schiano TD, Fiel MI, Crismale JF. Hemophagocytic lymphohistiocytosis occurring after liver transplantation: a case series and review of the literature. Clin Transpl. 2021;35(8):e14392.","journal-title":"Clin Transpl"},{"issue":"12","key":"2768_CR57","doi-asserted-by":"publisher","DOI":"10.1097\/TXD.0000000000000850","volume":"4","author":"GM Cohen","year":"2018","unstructured":"Cohen GM, Langer AL, Sima H, Chang C, Troy K, Taimur S. Hemophagocytic lymphohistiocytosis due to primary HHV-8 infection in a liver transplant recipient. Transpl Direct. 2018;4(12):e411.","journal-title":"Transpl Direct"},{"issue":"12","key":"2768_CR58","doi-asserted-by":"publisher","first-page":"3894","DOI":"10.1111\/ajt.16635","volume":"21","author":"LF Newell","year":"2021","unstructured":"Newell LF, Dunlap J, Gatter K, et al. Graft-versus-host disease after liver transplantation is associated with bone marrow failure, hemophagocytosis, and DNMT3A mutations. Am J Transpl. 2021;21(12):3894\u2013906.","journal-title":"Am J Transpl"},{"issue":"1","key":"2768_CR59","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1186\/s40792-018-0505-5","volume":"4","author":"N Iseda","year":"2018","unstructured":"Iseda N, Yoshizumi T, Toshima T, et al. Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literature. Surg Case Rep. 2018;4(1):101.","journal-title":"Surg Case Rep"},{"issue":"11","key":"2768_CR60","doi-asserted-by":"publisher","first-page":"2409","DOI":"10.1111\/trf.17096","volume":"62","author":"Z Xu","year":"2022","unstructured":"Xu Z, Otrock ZK. Extracorporeal photopheresis: a case of graft-versus-host-disease and hemophagocytic lymphohistiocytosis following liver transplantation. Transfusion. 2022;62(11):2409\u201313.","journal-title":"Transfusion"},{"issue":"4","key":"2768_CR61","doi-asserted-by":"publisher","first-page":"484","DOI":"10.1016\/j.mayocp.2013.12.012","volume":"89","author":"SA Parikh","year":"2014","unstructured":"Parikh SA, Kapoor P, Letendre L, Kumar S, Wolanskyj AP. Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis. Mayo Clin Proc. 2014;89(4):484\u201392.","journal-title":"Mayo Clin Proc"},{"issue":"22","key":"2768_CR62","doi-asserted-by":"publisher","first-page":"5794","DOI":"10.1182\/blood-2011-07-370148","volume":"118","author":"K Zhang","year":"2011","unstructured":"Zhang K, Jordan MB, Marsh RA, et al. Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH. Blood. 2011;118(22):5794\u20138.","journal-title":"Blood"},{"issue":"9","key":"2768_CR63","doi-asserted-by":"publisher","first-page":"2613","DOI":"10.1002\/art.38690","volume":"66","author":"L Fardet","year":"2014","unstructured":"Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613\u201320.","journal-title":"Arthritis Rheumatol"},{"issue":"6","key":"2768_CR64","doi-asserted-by":"publisher","first-page":"862","DOI":"10.1093\/ajcp\/aqw076","volume":"145","author":"F Debaugnies","year":"2016","unstructured":"Debaugnies F, Mahadeb B, Ferster A, et al. Performances of the H-score for diagnosis of hemophagocytic lymphohistiocytosis in adult and pediatric patients. Am J Clin Pathol. 2016;145(6):862\u201370.","journal-title":"Am J Clin Pathol"},{"issue":"2","key":"2768_CR65","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1111\/ejh.13779","volume":"109","author":"L Bilston","year":"2022","unstructured":"Bilston L, Croden J, Taparia M, Karkhaneh M, Grossman J, Sun HL. Validation of the HScore and the HLH-2004 diagnostic criteria for the diagnosis of hemophagocytic lymphohistiocytosis in a multicenter cohort. Eur J Haematol. 2022;109(2):129\u201337.","journal-title":"Eur J Haematol"},{"issue":"1","key":"2768_CR66","doi-asserted-by":"publisher","first-page":"244","DOI":"10.1186\/s13054-020-02941-3","volume":"24","author":"C Knaak","year":"2020","unstructured":"Knaak C, Nyvlt P, Schuster FS, et al. Hemophagocytic lymphohistiocytosis in critically ill patients: diagnostic reliability of HLH-2004 criteria and HScore. Crit Care. 2020;24(1):244.","journal-title":"Crit Care"},{"issue":"6","key":"2768_CR67","doi-asserted-by":"publisher","first-page":"1227","DOI":"10.1002\/pbc.21423","volume":"50","author":"CE Allen","year":"2008","unstructured":"Allen CE, Yu X, Kozinetz CA, McClain KL. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2008;50(6):1227\u201335.","journal-title":"Pediatr Blood Cancer"},{"issue":"10","key":"2768_CR68","doi-asserted-by":"publisher","first-page":"1548","DOI":"10.1182\/blood-2014-10-602607","volume":"125","author":"AM Schram","year":"2015","unstructured":"Schram AM, Campigotto F, Mullally A, et al. Marked hyperferritinemia does not predict for HLH in the adult population. Blood. 2015;125(10):1548\u201352.","journal-title":"Blood"},{"issue":"4","key":"2768_CR69","doi-asserted-by":"publisher","first-page":"344","DOI":"10.1111\/ejh.13391","volume":"104","author":"L Naymagon","year":"2020","unstructured":"Naymagon L, Tremblay D, Mascarenhas J. Reevaluating the role of ferritin in the diagnosis of adult secondary hemophagocytic lymphohistiocytosis. Eur J Haematol. 2020;104(4):344\u201351.","journal-title":"Eur J Haematol"},{"issue":"1","key":"2768_CR70","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1007\/s12291-017-0655-4","volume":"33","author":"S Basu","year":"2018","unstructured":"Basu S, Maji B, Barman S, Ghosh A. Hyperferritinemia in hemophagocytic lymphohistiocytosis: a single institution experience in pediatric patients. Indian J Clin Biochem. 2018;33(1):108\u201312.","journal-title":"Indian J Clin Biochem"},{"issue":"1","key":"2768_CR71","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1080\/07853890.2022.2027513","volume":"54","author":"Z Hua","year":"2022","unstructured":"Hua Z, He L, Zhang R, Liu M, Wang Z, Wang Y. Serum ferritin is a good indicator for predicting the efficacy of adult HLH induction therapy. Ann Med. 2022;54(1):283\u201392.","journal-title":"Ann Med"},{"issue":"1","key":"2768_CR72","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1007\/s00277-011-1239-7","volume":"91","author":"C Tabata","year":"2012","unstructured":"Tabata C, Tabata R. Possible prediction of underlying lymphoma by high sIL-2R\/ferritin ratio in hemophagocytic syndrome. Ann Hematol. 2012;91(1):63\u201371.","journal-title":"Ann Hematol"},{"issue":"5","key":"2768_CR73","doi-asserted-by":"publisher","first-page":"821","DOI":"10.1007\/s00277-013-1925-8","volume":"93","author":"T Tsuji","year":"2014","unstructured":"Tsuji T, Hirano T, Yamasaki H, Tsuji M, Tsuda H. A high sIL-2R\/ferritin ratio is a useful marker for the diagnosis of lymphoma-associated hemophagocytic syndrome. Ann Hematol. 2014;93(5):821\u20136.","journal-title":"Ann Hematol"},{"issue":"17","key":"2768_CR74","doi-asserted-by":"publisher","first-page":"4577","DOI":"10.1182\/blood-2011-06-356261","volume":"118","author":"H Trottestam","year":"2011","unstructured":"Trottestam H, Horne A, Aric\u00f2 M, et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood. 2011;118(17):4577\u201384.","journal-title":"Blood"},{"key":"2768_CR75","first-page":"95","volume":"14","author":"A Montecucco","year":"2015","unstructured":"Montecucco A, Zanetta F, Biamonti G. Molecular mechanisms of etoposide. EXCLI J. 2015;14:95\u2013108.","journal-title":"EXCLI J"},{"issue":"21","key":"2768_CR76","doi-asserted-by":"publisher","first-page":"1783","DOI":"10.1182\/blood.2019002289","volume":"134","author":"M Vallurupalli","year":"2019","unstructured":"Vallurupalli M, Berliner N. Emapalumab for the treatment of relapsed\/refractory hemophagocytic lymphohistiocytosis. Blood. 2019;134(21):1783\u20136.","journal-title":"Blood"},{"issue":"1","key":"2768_CR77","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1002\/pbc.24188","volume":"60","author":"RA Marsh","year":"2013","unstructured":"Marsh RA, Allen CE, McClain KL, et al. Salvage therapy of refractory hemophagocytic lymphohistiocytosis with alemtuzumab. Pediatr Blood Cancer. 2013;60(1):101\u20139.","journal-title":"Pediatr Blood Cancer"},{"issue":"7","key":"2768_CR78","doi-asserted-by":"publisher","first-page":"415","DOI":"10.1038\/nrclinonc.2010.40","volume":"7","author":"MP Strout","year":"2010","unstructured":"Strout MP, Seropian S, Berliner N. Alemtuzumab as a bridge to allogeneic SCT in atypical hemophagocytic lymphohistiocytosis. Nat Rev Clin Oncol. 2010;7(7):415\u201320.","journal-title":"Nat Rev Clin Oncol"},{"issue":"5","key":"2768_CR79","doi-asserted-by":"publisher","first-page":"e210","DOI":"10.3324\/haematol.2019.222471","volume":"105","author":"J Wang","year":"2020","unstructured":"Wang J, Wang Y, Wu L, et al. Ruxolitinib for refractory\/relapsed hemophagocytic lymphohistiocytosis. Haematologica. 2020;105(5):e210\u20132.","journal-title":"Haematologica"},{"issue":"23","key":"2768_CR80","doi-asserted-by":"publisher","first-page":"2465","DOI":"10.1182\/blood.2018894618","volume":"133","author":"P La Ros\u00e9e","year":"2019","unstructured":"La Ros\u00e9e P, Horne A, Hines M, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019;133(23):2465\u201377.","journal-title":"Blood"},{"issue":"6","key":"2768_CR81","doi-asserted-by":"publisher","first-page":"73","DOI":"10.5492\/wjccm.v7.i6.73","volume":"7","author":"S Kapoor","year":"2018","unstructured":"Kapoor S, Morgan CK, Siddique MA, Guntupalli KK. Intensive care unit complications and outcomes of adult patients with hemophagocytic lymphohistiocytosis: a retrospective study of 16 cases. World J Crit Care Med. 2018;7(6):73\u201383.","journal-title":"World J Crit Care Med"},{"issue":"51","key":"2768_CR82","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000002318","volume":"94","author":"T Barba","year":"2015","unstructured":"Barba T, Maucort-Boulch D, Iwaz J, et al. Hemophagocytic lymphohistiocytosis in intensive care unit: a 71-case strobe-compliant retrospective study. Medicine (Baltimore). 2015;94(51):e2318.","journal-title":"Medicine (Baltimore)"},{"issue":"2","key":"2768_CR83","doi-asserted-by":"publisher","first-page":"100","DOI":"10.1002\/hon.960","volume":"29","author":"M Shabbir","year":"2011","unstructured":"Shabbir M, Lucas J, Lazarchick J, Shirai K. Secondary hemophagocytic syndrome in adults: a case series of 18 patients in a single institution and a review of literature. Hematol Oncol. 2011;29(2):100\u20136.","journal-title":"Hematol Oncol"},{"issue":"6","key":"2768_CR84","doi-asserted-by":"publisher","first-page":"897","DOI":"10.1007\/s00277-011-1380-3","volume":"91","author":"HS Park","year":"2012","unstructured":"Park HS, Kim DY, Lee JH, et al. Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors. Ann Hematol. 2012;91(6):897\u2013904.","journal-title":"Ann Hematol"},{"issue":"1","key":"2768_CR85","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1093\/qjmed\/hcx183","volume":"111","author":"M Zhou","year":"2018","unstructured":"Zhou M, Li L, Zhang Q, et al. Clinical features and outcomes in secondary adult hemophagocytic lymphohistiocytosis. QJM. 2018;111(1):23\u201331.","journal-title":"QJM"},{"issue":"3","key":"2768_CR86","doi-asserted-by":"publisher","first-page":"852","DOI":"10.1016\/S0002-9270(00)02425-4","volume":"96","author":"C de Kerguenec","year":"2001","unstructured":"de Kerguenec C, Hillaire S, Molini\u00e9 V, et al. Hepatic manifestations of hemophagocytic syndrome: a study of 30 cases. Am J Gastroenterol. 2001;96(3):852\u20137.","journal-title":"Am J Gastroenterol"},{"issue":"2","key":"2768_CR87","doi-asserted-by":"publisher","first-page":"269","DOI":"10.3324\/haematol.2018.198655","volume":"104","author":"JH Yoon","year":"2019","unstructured":"Yoon JH, Park SS, Jeon YW, et al. Treatment outcomes and prognostic factors in adult patients with secondary hemophagocytic lymphohistiocytosis not associated with malignancy. Haematologica. 2019;104(2):269\u201376.","journal-title":"Haematologica"}],"container-title":["Advances in Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12325-023-02768-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s12325-023-02768-8\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12325-023-02768-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,2,4]],"date-time":"2024-02-04T07:22:40Z","timestamp":1707031360000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s12325-023-02768-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,12,25]]},"references-count":87,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2024,2]]}},"alternative-id":["2768"],"URL":"https:\/\/doi.org\/10.1007\/s12325-023-02768-8","relation":{},"ISSN":["0741-238X","1865-8652"],"issn-type":[{"value":"0741-238X","type":"print"},{"value":"1865-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,12,25]]},"assertion":[{"value":"7 November 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 December 2023","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 December 2023","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"No conflict of interest exists for all authors in this manuscript. All authors have approved the content of this manuscript. This manuscript has not been previously published and is not currently under consideration elsewhere for publication.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}},{"value":"This article is based on previously conducted studies and does not contain any new studies with human participants or animals performed by any of the authors.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Approval"}}]}}