{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,3]],"date-time":"2025-12-03T02:35:44Z","timestamp":1764729344063,"version":"3.46.0"},"reference-count":34,"publisher":"Springer Science and Business Media LLC","issue":"11","license":[{"start":{"date-parts":[[2025,11,7]],"date-time":"2025-11-07T00:00:00Z","timestamp":1762473600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"},{"start":{"date-parts":[[2025,11,7]],"date-time":"2025-11-07T00:00:00Z","timestamp":1762473600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82370185","82370185","82370185"],"award-info":[{"award-number":["82370185","82370185","82370185"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Science and Technology Special Project of Xicheng District","award":["XCSTS-TI2024-38","XCSTS-TI2024-38","XCSTS-TI2024-38"],"award-info":[{"award-number":["XCSTS-TI2024-38","XCSTS-TI2024-38","XCSTS-TI2024-38"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Ann Hematol"],"DOI":"10.1007\/s00277-025-06640-3","type":"journal-article","created":{"date-parts":[[2025,11,7]],"date-time":"2025-11-07T01:51:52Z","timestamp":1762480312000},"page":"5615-5624","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Monocyte\/macrophages may exert prominent roles in macrophage activation syndrome"],"prefix":"10.1007","volume":"104","author":[{"given":"Dongfei","family":"Yin","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jingshi","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhao","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,11,7]]},"reference":[{"key":"6640_CR1","doi-asserted-by":"publisher","first-page":"562","DOI":"10.1097\/BOR.0000000000000093","volume":"26","author":"LK Weaver","year":"2014","unstructured":"Weaver LK, Behrens EM (2014) Hyperinflammation, rather than hemophagocytosis, is the common link between macrophage activation syndrome and hemophagocytic lymphohistiocytosis. Curr Opin Rheumatol 26:562\u2013569","journal-title":"Curr Opin Rheumatol"},{"key":"6640_CR2","doi-asserted-by":"publisher","first-page":"940","DOI":"10.1016\/j.jaci.2020.07.025","volume":"146","author":"CW Freyer","year":"2020","unstructured":"Freyer CW, Porter DL (2020) Cytokine release syndrome and neurotoxicity following CAR T-cell therapy for hematologic malignancies. J Allergy Clin Immunol 146:940\u2013948","journal-title":"J Allergy Clin Immunol"},{"issue":"8","key":"6640_CR3","doi-asserted-by":"publisher","first-page":"822","DOI":"10.1016\/S2213-2600(20)30267-8","volume":"8","author":"P Mehta","year":"2020","unstructured":"Mehta P, Porter JC, Manson JJ, Isaacs JD, Openshaw P, Mcinnes IB et al (2020) Therapeutic blockade of granulocyte macrophage colony-stimulating factor in COVID-19-associated hyperinflammation: challenges and opportunities. Lancet Respir Med 8(8):822\u2013830","journal-title":"Lancet Respir Med"},{"key":"6640_CR4","doi-asserted-by":"crossref","unstructured":"Thwaites RS, Sanchez SUA, Siggins MK, Liew F, Russell CD, Moore SC et al (2021) Inflammatory profiles across the spectrum of disease reveal a distinct role for GM-CSF in severe COVID-19. Sci Immunol 6(57):eabg9873","DOI":"10.1126\/sciimmunol.abg9873"},{"issue":"6","key":"6640_CR5","doi-asserted-by":"publisher","first-page":"664","DOI":"10.1158\/2159-8290.CD-16-0040","volume":"6","author":"DT Teachey","year":"2016","unstructured":"Teachey DT, Lacey SF, Shaw PA, Melenhorst JJ, Maude SL, Frey N et al (2016) Identification of predictive biomarkers for cytokine release syndrome after chimeric antigen receptor T-cell therapy for acute lymphoblastic leukemia. Cancer Discov 6(6):664\u2013679","journal-title":"Cancer Discov"},{"key":"6640_CR6","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, Egeler RM, Filipovich AH, Imashuku S et al (2007) HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 48:124\u2013131","journal-title":"Pediatr Blood Cancer"},{"key":"6640_CR7","doi-asserted-by":"publisher","first-page":"2918","DOI":"10.1182\/blood.V78.11.2918.2918","volume":"78","author":"JI Henter","year":"1991","unstructured":"Henter JI, Elinder G, S\u00f6der O, Hansson M, Andersson B, Andersson U (1991) Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. Blood 78:2918\u20132922","journal-title":"Blood"},{"key":"6640_CR8","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1097\/MOP.0b013e32834ec9c1","volume":"24","author":"K Risma","year":"2012","unstructured":"Risma K, Jordan MB (2012) Hemophagocytic lymphohistiocytosis: updates and evolving concepts. Curr Opin Pediatr 24:9\u201315","journal-title":"Curr Opin Pediatr"},{"key":"6640_CR9","doi-asserted-by":"publisher","first-page":"735","DOI":"10.1182\/blood-2003-10-3413","volume":"104","author":"MB Jordan","year":"2004","unstructured":"Jordan MB, Hildeman D, Kappler J, Marrack P (2004) An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8\u2009+\u2009T cells and interferon gamma are essential for the disorder. Blood 104:735\u2013743","journal-title":"Blood"},{"key":"6640_CR10","doi-asserted-by":"publisher","first-page":"e88181","DOI":"10.1172\/jci.insight.88181","volume":"1","author":"M Wunderlich","year":"2016","unstructured":"Wunderlich M, Stockman C, Devarajan M, Ravishankar N, Sexton C, Kumar AR et al (2016) A xenograft model of macrophage activation syndrome amenable to anti-CD33 and anti-IL-6R treatment. JCI Insight 1:e88181","journal-title":"JCI Insight"},{"key":"6640_CR11","doi-asserted-by":"publisher","first-page":"2751","DOI":"10.1182\/bloodadvances.2020001624","volume":"4","author":"G Ingoglia","year":"2020","unstructured":"Ingoglia G, Yalamanoglu A, Pfefferl\u00e9 M, Dubach IL, Schaer CA, Valkova K et al (2020) Line-selective macrophage activation with an anti-CD40 antibody drives a hemophagocytic syndrome in mice. Blood Adv 4:2751\u20132761","journal-title":"Blood Adv"},{"key":"6640_CR12","doi-asserted-by":"publisher","first-page":"161","DOI":"10.1002\/art.40683","volume":"71","author":"LK Weaver","year":"2019","unstructured":"Weaver LK, Chu N, Behrens EM (2019) Brief report: Interferon-\u03b3-mediated immunopathology potentiated by Toll-like receptor 9 activation in a murine model of macrophage activation syndrome. Arthritis Rheumatol 71:161\u2013168","journal-title":"Arthritis Rheumatol"},{"key":"6640_CR13","doi-asserted-by":"publisher","first-page":"1442","DOI":"10.1182\/blood-2017-12-820852","volume":"131","author":"ES Weiss","year":"2018","unstructured":"Weiss ES, Girard-Guyonvarc\u2019H C, Holzinger D, de Jesus AA, Tariq Z, Picarsic J et al (2018) Interleukin-18 diagnostically distinguishes and pathogenically promotes human and murine macrophage activation syndrome. Blood 131:1442\u20131455","journal-title":"Blood"},{"key":"6640_CR14","doi-asserted-by":"publisher","first-page":"1507","DOI":"10.1093\/rheumatology\/keu524","volume":"54","author":"K Put","year":"2015","unstructured":"Put K, Avau A, Brisse E, Mitera T, Put S, Proost P et al (2015) Cytokines in systemic juvenile idiopathic arthritis and haemophagocytic lymphohistiocytosis: tipping the balance between interleukin-18 and interferon-\u03b3. Rheumatology (Oxford) 54:1507\u20131517","journal-title":"Rheumatology (Oxford)"},{"key":"6640_CR15","doi-asserted-by":"publisher","first-page":"e563","DOI":"10.1016\/S2665-9913(21)00115-6","volume":"3","author":"C Kessel","year":"2021","unstructured":"Kessel C, Fall N, Grom A, de Jager W, Vastert S, Strippoli R et al (2021) Definition and validation of serum biomarkers for optimal differentiation of hyperferritinaemic cytokine storm conditions in children: a retrospective cohort study. Lancet Rheumatol 3:e563\u2013e573","journal-title":"Lancet Rheumatol"},{"key":"6640_CR16","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1016\/j.berh.2014.03.002","volume":"28","author":"GS Schulert","year":"2014","unstructured":"Schulert GS, Grom AA (2014) Macrophage activation syndrome and cytokine-directed therapies. Best Pract Res Clin Rheumatol 28:277\u2013292","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"6640_CR17","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1136\/annrheumdis-2015-209020","volume":"76","author":"C Bracaglia","year":"2017","unstructured":"Bracaglia C, de Graaf K, Pires MD, Guilhot F, Ferlin W, Prencipe G et al (2017) Elevated Circulating levels of interferon-\u03b3 and interferon-\u03b3-induced chemokines characterise patients with macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. Ann Rheum Dis 76:166\u2013172","journal-title":"Ann Rheum Dis"},{"key":"6640_CR18","doi-asserted-by":"publisher","first-page":"2186","DOI":"10.1182\/blood-2015-05-644914","volume":"126","author":"Y Wang","year":"2015","unstructured":"Wang Y, Huang W, Hu L, Cen X, Li L, Wang J et al (2015) Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis. Blood 126:2186\u20132192","journal-title":"Blood"},{"key":"6640_CR19","doi-asserted-by":"publisher","first-page":"89","DOI":"10.1007\/s12519-019-00256-0","volume":"16","author":"LX Zou","year":"2020","unstructured":"Zou LX, Zhu Y, Sun L, Ma HH, Yang SR, Zeng HS et al (2020) Clinical and laboratory features, treatment, and outcomes of macrophage activation syndrome in 80 children: a multi-center study in China. World J Pediatr 16:89\u201398","journal-title":"World J Pediatr"},{"key":"6640_CR20","doi-asserted-by":"publisher","first-page":"743","DOI":"10.1016\/j.autrev.2017.05.010","volume":"16","author":"PE Gavand","year":"2017","unstructured":"Gavand PE, Serio I, Arnaud L, Costedoat-Chalumeau N, Carvelli J, Dossier A et al (2017) Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: a study of 103 episodes in 89 adult patients. Autoimmun Rev 16:743\u2013749","journal-title":"Autoimmun Rev"},{"key":"6640_CR21","doi-asserted-by":"publisher","first-page":"965","DOI":"10.1002\/art.22416","volume":"56","author":"J Bleesing","year":"2007","unstructured":"Bleesing J, Prada A, Siegel DM, Villanueva J, Olson J, Ilowite NT et al (2007) The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor alpha-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis. Arthritis Rheum 56:965\u2013971","journal-title":"Arthritis Rheum"},{"key":"6640_CR22","doi-asserted-by":"publisher","first-page":"285","DOI":"10.1002\/art.42680","volume":"76","author":"KE Brodeur","year":"2024","unstructured":"Brodeur KE, Liu M, Ibanez D, de Groot MJ, Chen L, Du Y et al (2024) Elevation of IL-17 cytokines distinguishes Kawasaki disease from other pediatric inflammatory disorders. Arthritis Rheumatol 76:285\u2013292","journal-title":"Arthritis Rheumatol"},{"key":"6640_CR23","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1093\/rheumatology\/keac247","volume":"62","author":"P Ruscitti","year":"2022","unstructured":"Ruscitti P, Ursini F, Berardicurti O, Masedu F, Bozzalla CE, Naldi S et al (2022) Cytokine profile, ferritin and multi-visceral involvement characterize macrophage activation syndrome during adult-onset still\u2019s disease. Rheumatology (Oxford) 62:321\u2013329","journal-title":"Rheumatology (Oxford)"},{"key":"6640_CR24","doi-asserted-by":"publisher","first-page":"3349","DOI":"10.2217\/fon-2022-0063","volume":"18","author":"KS Rallis","year":"2022","unstructured":"Rallis KS, Corrigan AE, Dadah H, Stanislovas J, Zamani P, Makker S et al (2022) IL-10 in cancer: an essential thermostatic regulator between homeostatic immunity and inflammation - a comprehensive review. Future Oncol 18:3349\u20133365","journal-title":"Future Oncol"},{"key":"6640_CR25","doi-asserted-by":"publisher","first-page":"2255","DOI":"10.1056\/NEJMra2026131","volume":"383","author":"DC Fajgenbaum","year":"2020","unstructured":"Fajgenbaum DC, June CH (2020) Cytokine storm. N Engl J Med 383:2255\u20132273","journal-title":"N Engl J Med"},{"key":"6640_CR26","doi-asserted-by":"publisher","first-page":"310","DOI":"10.1002\/hon.2283","volume":"35","author":"HS Kim","year":"2017","unstructured":"Kim HS, Ryu KJ, Ko YH, Kim HJ, Kim SH, Kim WS et al (2017) Macrophage inflammatory protein 1 alpha (MIP-1alpha) May be associated with poor outcome in patients with extranodal NK\/T-cell lymphoma. Hematol Oncol 35:310\u2013316","journal-title":"Hematol Oncol"},{"key":"6640_CR27","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1038\/nrrheum.2015.179","volume":"12","author":"AA Grom","year":"2016","unstructured":"Grom AA, Horne A, De Benedetti F (2016) Macrophage activation syndrome in the era of biologic therapy. Nat Rev Rheumatol 12:259\u2013268","journal-title":"Nat Rev Rheumatol"},{"key":"6640_CR28","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1007\/s10875-024-01797-4","volume":"45","author":"HA Carol","year":"2024","unstructured":"Carol HA, Mayer AS, Zhang MS, Dang V, Varghese J, Martinez Z et al (2024) Hyperferritinemia screening to aid identification and differentiation of patients with hyperinflammatory disorders. J Clin Immunol 45:4","journal-title":"J Clin Immunol"},{"key":"6640_CR29","doi-asserted-by":"publisher","first-page":"2264","DOI":"10.1172\/JCI43157","volume":"121","author":"EM Behrens","year":"2011","unstructured":"Behrens EM, Canna SW, Slade K, Rao S, Kreiger PA, Paessler M et al (2011) Repeated TLR9 stimulation results in macrophage activation syndrome-like disease in mice. J Clin Invest 121:2264\u20132277","journal-title":"J Clin Invest"},{"key":"6640_CR30","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1002\/art.41076","volume":"72","author":"TN Burn","year":"2020","unstructured":"Burn TN, Weaver L, Rood JE, Chu N, Bodansky A, Kreiger PA et al (2020) Genetic deficiency of interferon-\u03b3 reveals interferon-\u03b3-independent manifestations of murine hemophagocytic lymphohistiocytosis. Arthritis Rheumatol 72:335\u2013347","journal-title":"Arthritis Rheumatol"},{"key":"6640_CR31","doi-asserted-by":"publisher","first-page":"1645","DOI":"10.1093\/rheumatology\/keq133","volume":"49","author":"M Shimizu","year":"2010","unstructured":"Shimizu M, Yokoyama T, Yamada K, Kaneda H, Wada H, Wada T et al (2010) Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis. Rheumatology (Oxford) 49:1645\u20131653","journal-title":"Rheumatology (Oxford)"},{"key":"6640_CR32","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1084\/jem.20041257","volume":"201","author":"CL Langrish","year":"2005","unstructured":"Langrish CL, Chen Y, Blumenschein WM, Mattson J, Basham B, Sedgwick JD et al (2005) IL-23 drives a pathogenic T cell population that induces autoimmune inflammation. J Exp Med 201:233\u2013240","journal-title":"J Exp Med"},{"issue":"1","key":"6640_CR33","doi-asserted-by":"publisher","first-page":"102","DOI":"10.15252\/embj.201694615","volume":"36","author":"IA Mufazalov","year":"2017","unstructured":"Mufazalov IA, Schelmbauer C, Regen T, Kuschmann J, Wanke F, Gabriel LA et al (2017) <scp>IL<\/scp>\u20101 signaling is critical for expansion but not generation of autoreactive <scp>GM<\/scp>\u2010 <scp>CSF<\/scp>+ Th17 cells. EMBO J 36(1):102\u2013115","journal-title":"EMBO J"},{"key":"6640_CR34","doi-asserted-by":"publisher","first-page":"1340","DOI":"10.1002\/art.38359","volume":"66","author":"A Avau","year":"2014","unstructured":"Avau A, Mitera T, Put S, Put K, Brisse E, Filtjens J et al (2014) Systemic juvenile idiopathic arthritis\u2013like syndrome in mice following stimulation of the immune system with Freund\u2019s complete adjuvant: regulation by interferon\u2010\u03b3. Arthritis Rheumatol 66:1340\u20131351","journal-title":"Arthritis Rheumatol"}],"container-title":["Annals of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00277-025-06640-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00277-025-06640-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00277-025-06640-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,3]],"date-time":"2025-12-03T02:31:26Z","timestamp":1764729086000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00277-025-06640-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,7]]},"references-count":34,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2025,11]]}},"alternative-id":["6640"],"URL":"https:\/\/doi.org\/10.1007\/s00277-025-06640-3","relation":{},"ISSN":["1432-0584"],"issn-type":[{"type":"electronic","value":"1432-0584"}],"subject":[],"published":{"date-parts":[[2025,11,7]]},"assertion":[{"value":"14 May 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 September 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 November 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}},{"value":"There was no conflict of interest in this article.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}