{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T15:38:25Z","timestamp":1777045105421,"version":"3.51.4"},"reference-count":143,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2025,6,1]],"date-time":"2025-06-01T00:00:00Z","timestamp":1748736000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,6,1]],"date-time":"2025-06-01T00:00:00Z","timestamp":1748736000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,6,1]],"date-time":"2025-06-01T00:00:00Z","timestamp":1748736000000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2025,6,1]],"date-time":"2025-06-01T00:00:00Z","timestamp":1748736000000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2025,6,1]],"date-time":"2025-06-01T00:00:00Z","timestamp":1748736000000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2025,6,1]],"date-time":"2025-06-01T00:00:00Z","timestamp":1748736000000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2025,6,1]],"date-time":"2025-06-01T00:00:00Z","timestamp":1748736000000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/100017655","name":"Incyte","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100017655","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100012524","name":"ALSAC","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100012524","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.fr","clinicalkey.jp","theclinics.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Hematology\/Oncology Clinics of North America"],"published-print":{"date-parts":[[2025,6]]},"DOI":"10.1016\/j.hoc.2025.02.003","type":"journal-article","created":{"date-parts":[[2025,3,25]],"date-time":"2025-03-25T13:35:23Z","timestamp":1742909723000},"page":"553-575","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":3,"title":["Clinical Characteristics and Treatment of Familial Hemophagocytic Lymphohistiocytosis"],"prefix":"10.1016","volume":"39","author":[{"given":"Lauren K.","family":"Meyer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Camille","family":"Keenan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kim E.","family":"Nichols","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"issue":"4","key":"10.1016\/j.hoc.2025.02.003_bib1","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1111\/j.1651-2227.1991.tb11878.x","article-title":"Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis","volume":"80","author":"Henter","year":"1991","journal-title":"Acta Paediatr Scand"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib2","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1002\/pbc.22359","article-title":"Hemophagocytic lymphohistiocytosis in Texas: observations on ethnicity and race","volume":"54","author":"Niece","year":"2010","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.hoc.2025.02.003_bib3","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2021.777851","article-title":"Digenic inheritance: evidence and gaps in hemophagocytic lymphohistiocytosis","volume":"12","author":"Steen","year":"2021","journal-title":"Front Immunol"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib143","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1086\/302187","article-title":"Localization of a gene for familial hemophagocytic lymphohistiocytosis at chromosome 9q21.3-22 by homozygosity mapping","volume":"64","author":"Ohadi","year":"1999","journal-title":"Am J Hum Genet"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib4","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1007\/BF00443367","article-title":"Familial hemophagocytic lymphohistiocytosis","volume":"140","author":"Janka","year":"1983","journal-title":"Eur J Pediatr"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib5","first-page":"197","article-title":"Hemophagocytic lymphohistiocytosis. Report of 122 children from the international registry. FHL study group of the histiocyte society","volume":"10","author":"Aric\u00f2","year":"1996","journal-title":"Leukemia"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib6","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1007\/s00431-006-0258-1","article-title":"Familial and acquired hemophagocytic lymphohistiocytosis","volume":"166","author":"Janka","year":"2007","journal-title":"Eur J Pediatr"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib7","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1111\/ejh.12399","article-title":"Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in Korea: report of a nationwide survey from Korea Histiocytosis Working Party","volume":"94","author":"Koh","year":"2015","journal-title":"Eur J Haematol"},{"key":"10.1016\/j.hoc.2025.02.003_bib8","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2021.612583","article-title":"The spectrum of clinical, immunological, and molecular findings in familial hemophagocytic lymphohistiocytosis: experience from India","volume":"12","author":"Shabrish","year":"2021","journal-title":"Front Immunol"},{"issue":"5","key":"10.1016\/j.hoc.2025.02.003_bib9","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1097\/MPH.0000000000002653","article-title":"Prognostic factors and long-term outcomes in 41 children with primary hemophagocytic lymphohistiocytosis: report of a single-center experience and review of the literature","volume":"45","author":"Bayram","year":"2023","journal-title":"J Pediatr Hematol Oncol"},{"issue":"8","key":"10.1016\/j.hoc.2025.02.003_bib10","first-page":"2515","article-title":"Outcome of primary hemophagocytic lymphohistiocytosis: a report on 143 patients from the Italian Registry","volume":"109","author":"Pegoraro","year":"2024","journal-title":"Haematologica"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib11","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1007\/s00277-023-05499-6","article-title":"Genetic and clinical characteristics of primary hemophagocytic lymphohistiocytosis in children","volume":"103","author":"Zhao","year":"2024","journal-title":"Ann Hematol"},{"issue":"4","key":"10.1016\/j.hoc.2025.02.003_bib12","doi-asserted-by":"crossref","first-page":"398","DOI":"10.5152\/TurkArchPediatr.2022.21314","article-title":"Clinical, genetic, and outcome characteristics of pediatric patients with primary hemophagocytic lymphohistiocytosis","volume":"57","author":"Nepesov","year":"2022","journal-title":"Turk Arch Pediatr"},{"issue":"1 Pt 1","key":"10.1016\/j.hoc.2025.02.003_bib13","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1016\/j.jfma.2020.03.025","article-title":"Outcome analysis of pediatric hemophagocytic lymphohistiocytosis","volume":"120","author":"Chen","year":"2021","journal-title":"J Formos Med Assoc Taiwan Yi Zhi"},{"issue":"10","key":"10.1016\/j.hoc.2025.02.003_bib14","doi-asserted-by":"crossref","first-page":"2723","DOI":"10.1111\/apa.15889","article-title":"Clinical features, diagnosis and therapy of familial haemophagocytic lymphohistiocytosis","volume":"110","author":"Janka","year":"2021","journal-title":"Acta Paediatr"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib15","doi-asserted-by":"crossref","first-page":"e217","DOI":"10.1097\/MPH.0000000000002283","article-title":"Clinical features and prognostic factors of early outcome in pediatric hemophagocytic lymphohistiocytosis: a retrospective analysis of 227 cases","volume":"44","author":"Zhou","year":"2022","journal-title":"J Pediatr Hematol Oncol"},{"key":"10.1016\/j.hoc.2025.02.003_bib16","author":"Zhang"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib17","doi-asserted-by":"crossref","first-page":"231","DOI":"10.3324\/haematol.2017.178038","article-title":"The clinical and laboratory evaluation of familial hemophagocytic lymphohistiocytosis and the importance of hepatic and spinal cord involvement: a single center experience","volume":"103","author":"Beken","year":"2018","journal-title":"Haematologica"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib18","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1007\/s10875-020-00814-6","article-title":"Neuroinflammatory disease as an isolated manifestation of hemophagocytic lymphohistiocytosis","volume":"40","author":"Blincoe","year":"2020","journal-title":"J Clin Immunol"},{"issue":"10","key":"10.1016\/j.hoc.2025.02.003_bib19","doi-asserted-by":"crossref","first-page":"2303","DOI":"10.1007\/s00277-019-03764-1","article-title":"Genetic characterization of pediatric primary hemophagocytic lymphohistiocytosis in China: a single-center study","volume":"98","author":"Zhang","year":"2019","journal-title":"Ann Hematol"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib20","doi-asserted-by":"crossref","first-page":"159.e9-e17","DOI":"10.1016\/j.crad.2020.09.009","article-title":"MRI findings of central nervous system involvement in children with haemophagocytic lymphohistiocytosis: correlation with clinical biochemical tests","volume":"76","author":"Ma","year":"2021","journal-title":"Clin Radiol"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib21","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/j.clinimag.2019.10.001","article-title":"Neuroimaging features of CNS histiocytosis syndromes","volume":"60","author":"Wang","year":"2020","journal-title":"Clin Imaging"},{"issue":"4","key":"10.1016\/j.hoc.2025.02.003_bib22","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1055\/s-0043-1772495","article-title":"Imaging spectrum of neurological manifestations of hemophagocytic lymphohistiocytosis in pediatrics: a case series","volume":"33","author":"Sivaramalingam","year":"2023","journal-title":"Indian J Radiol Imaging"},{"issue":"10","key":"10.1016\/j.hoc.2025.02.003_bib23","doi-asserted-by":"crossref","first-page":"7149","DOI":"10.1007\/s00330-023-09649-2","article-title":"Spectrum of neuroradiological manifestations in primary hemophagocytic lymphohistiocytosis: a comparative study of EBV-induced versus non-EBV-induced forms in 75 genetically confirmed pediatric cases","volume":"33","author":"Charpentier","year":"2023","journal-title":"Eur Radiol"},{"issue":"12","key":"10.1016\/j.hoc.2025.02.003_bib24","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1093\/jnen\/nly094","article-title":"Histopathologic correlates of familial hemophagocytic lymphohistiocytosis isolated to the central nervous system","volume":"77","author":"Solomon","year":"2018","journal-title":"J Neuropathol Exp Neurol"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib25","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1093\/jnen\/nlz001","article-title":"A review of neuropathological features of familial and adult hemophagocytic lymphohistiocytosis","volume":"78","author":"Klein","year":"2019","journal-title":"J Neuropathol Exp Neurol"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib26","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1097\/MPG.0000000000003807","article-title":"The liver in hemophagocytic lymphohistiocytosis: not an innocent bystander","volume":"77","author":"Diamond","year":"2023","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"10.1016\/j.hoc.2025.02.003_bib27","doi-asserted-by":"crossref","first-page":"227","DOI":"10.2147\/JBM.S92438","article-title":"A retrospective analysis of 56 children with hemophagocytic lymphohistiocytosis","volume":"7","author":"Wang","year":"2016","journal-title":"J Blood Med"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib28","doi-asserted-by":"crossref","DOI":"10.4084\/mjhid.2014.067","article-title":"Importance of hyperbilirubinemia in differentiation of primary and secondary hemophagocytic lymphohistiocytosis in pediatric cases","volume":"6","author":"Ozen","year":"2014","journal-title":"Mediterr J Hematol Infect Dis"},{"key":"10.1016\/j.hoc.2025.02.003_bib29","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.jpeds.2022.07.006","article-title":"High prevalence of hemophagocytic lymphohistiocytosis in acute liver failure of infancy","volume":"250","author":"Had\u017ei\u0107","year":"2022","journal-title":"J Pediatr"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib30","doi-asserted-by":"crossref","first-page":"748","DOI":"10.1111\/ped.12181","article-title":"Differential clinical characteristics of acute liver failure caused by hemophagocytic lymphohistiocytosis in children","volume":"55","author":"Ryu","year":"2013","journal-title":"Pediatr Int"},{"issue":"4","key":"10.1016\/j.hoc.2025.02.003_bib31","first-page":"e185","article-title":"Outcomes analysis of children diagnosed with hemophagocytic lymphohistiocytosis in the PICU","volume":"20","author":"Gregory","year":"2019","journal-title":"Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensive Crit Care Soc"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib32","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.30122","article-title":"Treatment and mortality of hemophagocytic lymphohistiocytosis in critically ill children: a systematic review and meta-analysis","volume":"70","author":"Tan","year":"2023","journal-title":"Pediatr Blood Cancer"},{"issue":"9","key":"10.1016\/j.hoc.2025.02.003_bib33","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1001\/archderm.138.9.1208","article-title":"Cutaneous manifestations of hemophagocytic lymphohistiocytosis","volume":"138","author":"Morrell","year":"2002","journal-title":"Arch Dermatol"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib34","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1097\/MPH.0b013e3181923cb4","article-title":"Experience with hemophagocytic lymphohistiocytosis\/macrophage activation syndrome at a single institution","volume":"31","author":"Gupta","year":"2009","journal-title":"J Pediatr Hematol Oncol"},{"issue":"5","key":"10.1016\/j.hoc.2025.02.003_bib35","doi-asserted-by":"crossref","first-page":"860","DOI":"10.1097\/CCM.0000000000005361","article-title":"Consensus-based guidelines for the recognition, diagnosis, and management of hemophagocytic lymphohistiocytosis in critically ill children and adults","volume":"50","author":"Hines","year":"2022","journal-title":"Crit Care Med"},{"key":"10.1016\/j.hoc.2025.02.003_bib36","first-page":"1","article-title":"Inborn errors of immunity in early childhood: essential insights for the neonatologist","author":"Dirks","year":"2024","journal-title":"Neonatology"},{"issue":"4","key":"10.1016\/j.hoc.2025.02.003_bib37","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.30894","article-title":"Neonatal hemophagocytic lymphohistiocytosis: a meta-analysis of 205 cases","volume":"71","author":"Kranz","year":"2024","journal-title":"Pediatr Blood Cancer"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib38","doi-asserted-by":"crossref","first-page":"e26","DOI":"10.1097\/MPG.0000000000002554","article-title":"Liver failure among young Saudi infants: etiology, clinical presentation, and outcome","volume":"70","author":"Lone","year":"2020","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib39","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1097\/MPG.0000000000003103","article-title":"Applying an age-specific definition to better characterize etiologies and outcomes in neonatal acute liver failure","volume":"73","author":"Borovsky","year":"2021","journal-title":"J Pediatr Gastroenterol Nutr"},{"issue":"11","key":"10.1016\/j.hoc.2025.02.003_bib40","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.27344","article-title":"Is an infectious trigger always required for primary hemophagocytic lymphohistiocytosis? Lessons from in utero and neonatal disease","volume":"65","author":"Heeg","year":"2018","journal-title":"Pediatr Blood Cancer"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib41","doi-asserted-by":"crossref","first-page":"e316","DOI":"10.1542\/neo.20-6-e316","article-title":"Neonatal hemophagocytic lymphohistiocytosis","volume":"20","author":"McLean","year":"2019","journal-title":"NeoReviews"},{"key":"10.1016\/j.hoc.2025.02.003_bib42","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1186\/1750-1172-8-168","article-title":"Clinical, laboratory and molecular signs of immunodeficiency in patients with partial oculo-cutaneous albinism","volume":"8","author":"Dotta","year":"2013","journal-title":"Orphanet J Rare Dis"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib43","first-page":"140","article-title":"Hemophagocytic lymphohistiocytosis in children with Griscelli syndrome type 2: genetics, laboratory findings and treatment","volume":"12","author":"Cay","year":"2023","journal-title":"Am J Clin Exp Immunol"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib44","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1038\/76024","article-title":"Mutations in RAB27A cause Griscelli syndrome associated with haemophagocytic syndrome","volume":"25","author":"M\u00e9nasch\u00e9","year":"2000","journal-title":"Nat Genet"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib45","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1007\/BF03402003","article-title":"The Chediak-Higashi protein interacts with SNARE complex and signal transduction proteins","volume":"8","author":"Tchernev","year":"2002","journal-title":"Mol Med Camb Mass"},{"key":"10.1016\/j.hoc.2025.02.003_bib46","series-title":"GeneReviews\u00ae","article-title":"Chediak-higashi syndrome","author":"Toro","year":"1993"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib47","first-page":"362","article-title":"[New leukocyte anomaly of constitutional and familial character]","volume":"7","author":"Chediak","year":"1952","journal-title":"Rev Hematol"},{"issue":"4","key":"10.1016\/j.hoc.2025.02.003_bib48","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1097\/00005792-197207000-00001","article-title":"The Chediak-Higashi syndrome: studies in four patients and a review of the literature","volume":"51","author":"Blume","year":"1972","journal-title":"Medicine (Baltim)"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib49","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1006\/mgme.1999.2927","article-title":"Clinical, molecular, and cell biological aspects of Chediak-Higashi syndrome","volume":"68","author":"Introne","year":"1999","journal-title":"Mol Genet Metab"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib50","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1182\/blood-2005-11-4413","article-title":"Lethal hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type II","volume":"108","author":"Enders","year":"2006","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib51","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1111\/bjh.15274","article-title":"How i treat primary haemophagocytic lymphohistiocytosis","volume":"182","author":"Marsh","year":"2018","journal-title":"Br J Haematol"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib52","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":"10.1016\/j.hoc.2025.02.003_bib53","doi-asserted-by":"crossref","DOI":"10.1182\/blood.2024025077","article-title":"Diagnostic guidelines for familial hemophagocytic lymphohistiocytosis revisited","author":"Henter","year":"2024","journal-title":"Blood"},{"issue":"9","key":"10.1016\/j.hoc.2025.02.003_bib54","doi-asserted-by":"crossref","first-page":"2613","DOI":"10.1002\/art.38690","article-title":"Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome","volume":"66","author":"Fardet","year":"2014","journal-title":"Arthritis Rheumatol Hoboken NJ"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib55","doi-asserted-by":"crossref","first-page":"1227","DOI":"10.1002\/pbc.21423","article-title":"Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis","volume":"50","author":"Allen","year":"2008","journal-title":"Pediatr Blood Cancer"},{"issue":"11","key":"10.1016\/j.hoc.2025.02.003_bib56","doi-asserted-by":"crossref","first-page":"2101","DOI":"10.1002\/pbc.25058","article-title":"Determination of an appropriate cut-off value for ferritin in the diagnosis of hemophagocytic lymphohistiocytosis","volume":"61","author":"Lehmberg","year":"2014","journal-title":"Pediatr Blood Cancer"},{"issue":"8","key":"10.1016\/j.hoc.2025.02.003_bib57","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1007\/s00277-017-2993-y","article-title":"Clinical utility of soluble interleukin-2 receptor in hemophagocytic syndromes: a systematic scoping review","volume":"96","author":"Lin","year":"2017","journal-title":"Ann Hematol"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib58","doi-asserted-by":"crossref","first-page":"1245","DOI":"10.1016\/j.jpeds.2012.11.081","article-title":"Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis","volume":"162","author":"Lehmberg","year":"2013","journal-title":"J Pediatr"},{"issue":"9","key":"10.1016\/j.hoc.2025.02.003_bib59","article-title":"Genetic features of late onset primary hemophagocytic lymphohistiocytosis in adolescence or adulthood","volume":"9","author":"Wang","year":"2014","journal-title":"PLoS One"},{"key":"10.1016\/j.hoc.2025.02.003_bib60","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1186\/s13073-015-0244-1","article-title":"Targeted high-throughput sequencing for genetic diagnostics of hemophagocytic lymphohistiocytosis","volume":"7","author":"Tesi","year":"2015","journal-title":"Genome Med"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib61","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1111\/cge.13363","article-title":"Genetic variant spectrum in 265 Chinese patients with hemophagocytic lymphohistiocytosis: molecular analyses of PRF1, UNC13D, STX11, STXBP2, SH2D1A, and XIAP","volume":"94","author":"Chen","year":"2018","journal-title":"Clin Genet"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib62","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1007\/s00005-021-00635-4","article-title":"Molecular genetics diversity of primary hemophagocytic lymphohistiocytosis among polish pediatric patients","volume":"69","author":"B\u0105bol-Pokora","year":"2021","journal-title":"Arch Immunol Ther Exp (Warsz)"},{"issue":"12","key":"10.1016\/j.hoc.2025.02.003_bib63","doi-asserted-by":"crossref","first-page":"2578","DOI":"10.1182\/bloodadvances.2020001605","article-title":"Frequency and spectrum of disease-causing variants in 1892 patients with suspected genetic HLH disorders","volume":"4","author":"Gadoury-Levesque","year":"2020","journal-title":"Blood Adv"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib64","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1182\/blood-2017-11-814244","article-title":"Genetic and mechanistic diversity in pediatric hemophagocytic lymphohistiocytosis","volume":"132","author":"Chinn","year":"2018","journal-title":"Blood"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib65","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1097\/ACI.0b013e3283588ca6","article-title":"Use of whole exome and genome sequencing in the identification of genetic causes of primary immunodeficiencies","volume":"12","author":"Chou","year":"2012","journal-title":"Curr Opin Allergy Clin Immunol"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib66","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1016\/j.jaci.2015.06.048","article-title":"Genetic predisposition to hemophagocytic lymphohistiocytosis: report on 500 patients from the Italian registry","volume":"137","author":"Cetica","year":"2016","journal-title":"J Allergy Clin Immunol"},{"issue":"8","key":"10.1016\/j.hoc.2025.02.003_bib67","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1007\/s10875-017-0443-1","article-title":"Effective immunological guidance of genetic analyses including exome sequencing in patients evaluated for hemophagocytic lymphohistiocytosis","volume":"37","author":"Ammann","year":"2017","journal-title":"J Clin Immunol"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib68","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1186\/s13023-020-01390-z","article-title":"Genotype characteristics and immunological indicator evaluation of 311 hemophagocytic lymphohistiocytosis cases in China","volume":"15","author":"Zhang","year":"2020","journal-title":"Orphanet J Rare Dis"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib69","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1182\/blood.V99.1.61","article-title":"Perforin expression in cytotoxic lymphocytes from patients with hemophagocytic lymphohistiocytosis and their family members","volume":"99","author":"Kogawa","year":"2002","journal-title":"Blood"},{"issue":"15","key":"10.1016\/j.hoc.2025.02.003_bib70","doi-asserted-by":"crossref","first-page":"1858","DOI":"10.1182\/blood-2015-06-648659","article-title":"Accuracy of flow cytometric perforin screening for detecting patients with FHL due to PRF1 mutations","volume":"126","author":"Abdalgani","year":"2015","journal-title":"Blood"},{"issue":"1\u20132","key":"10.1016\/j.hoc.2025.02.003_bib71","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.jim.2004.08.008","article-title":"CD107a as a functional marker for the identification of natural killer cell activity","volume":"294","author":"Alter","year":"2004","journal-title":"J Immunol Methods"},{"key":"10.1016\/j.hoc.2025.02.003_bib72","article-title":"Efficacy of T cell assays for the diagnosis of primary defects in cytotoxic lymphocyte exocytosis","author":"Chiang","year":"2024","journal-title":"Blood"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib73","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1111\/j.1365-2141.2010.08289.x","article-title":"A novel assay for investigation of suspected familial haemophagocytic lymphohistiocytosis","volume":"150","author":"Wheeler","year":"2010","journal-title":"Br J Haematol"},{"key":"10.1016\/j.hoc.2025.02.003_bib74","doi-asserted-by":"crossref","DOI":"10.1155\/2016\/3769590","article-title":"A modified NK cell degranulation assay applicable for routine evaluation of NK cell function","volume":"2016","author":"Shabrish","year":"2016","journal-title":"J Immunol Res"},{"key":"10.1016\/j.hoc.2025.02.003_bib75","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2024.1391967","article-title":"A degranulation assay using V\u03b39V\u03b42 T cells for the rapid diagnosis of familial hemophagocytic syndromes","volume":"15","author":"Jorisch-M\u00fchlebach","year":"2024","journal-title":"Front Immunol"},{"issue":"12","key":"10.1016\/j.hoc.2025.02.003_bib76","doi-asserted-by":"crossref","first-page":"2754","DOI":"10.1182\/blood-2011-08-374199","article-title":"A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes","volume":"119","author":"Bryceson","year":"2012","journal-title":"Blood"},{"issue":"22","key":"10.1016\/j.hoc.2025.02.003_bib77","doi-asserted-by":"crossref","first-page":"2993","DOI":"10.1182\/blood-2016-12-753830","article-title":"Perforin and CD107a testing is superior to NK cell function testing for screening patients for genetic HLH","volume":"129","author":"Rubin","year":"2017","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib78","first-page":"181","article-title":"Quantitative assay of the lytic action of immune lymphoid cells on 51-Cr-labelled allogeneic target cells in vitro; inhibition by isoantibody and by drugs","volume":"14","author":"Brunner","year":"1968","journal-title":"Immunology"},{"issue":"1\u20132","key":"10.1016\/j.hoc.2025.02.003_bib79","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/j.jim.2005.06.005","article-title":"A sensitive flow cytometry-based cytotoxic T-lymphocyte assay through detection of cleaved caspase 3 in target cells","volume":"304","author":"He","year":"2005","journal-title":"J Immunol Methods"},{"key":"10.1016\/j.hoc.2025.02.003_bib80","first-page":"7.42.1","article-title":"A real-time cytotoxicity assay as an alternative to the standard chromium-51 release assay for measurement of human NK and T cell cytotoxic activity","volume":"118","author":"Fassy","year":"2017","journal-title":"Curr Protoc Immunol"},{"key":"10.1016\/j.hoc.2025.02.003_bib81","doi-asserted-by":"crossref","first-page":"1740","DOI":"10.3389\/fimmu.2019.01740","article-title":"Current flow cytometric assays for the screening and diagnosis of primary HLH","volume":"10","author":"Chiang","year":"2019","journal-title":"Front Immunol"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib82","doi-asserted-by":"crossref","first-page":"984","DOI":"10.1016\/j.jpeds.2011.11.046","article-title":"Diagnostic accuracy of a specific cytokine pattern in hemophagocytic lymphohistiocytosis in children","volume":"160","author":"Xu","year":"2012","journal-title":"J Pediatr"},{"issue":"5","key":"10.1016\/j.hoc.2025.02.003_bib83","doi-asserted-by":"crossref","first-page":"1592","DOI":"10.1016\/j.jaci.2022.02.028","article-title":"Distinguishing immune activation and inflammatory signatures of multisystem inflammatory syndrome in children (MIS-C) versus hemophagocytic lymphohistiocytosis (HLH)","volume":"149","author":"Kumar","year":"2022","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.hoc.2025.02.003_bib84","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1007\/978-3-031-59815-9_13","article-title":"Cytokines in cytokine Storm syndrome","volume":"1448","author":"Behrens","year":"2024","journal-title":"Adv Exp Med Biol"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib85","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/j.jri.2005.03.005","article-title":"Validation and comparison of luminex multiplex cytokine analysis kits with ELISA: determinations of a panel of nine cytokines in clinical sample culture supernatants","volume":"66","author":"dupont","year":"2005","journal-title":"J Reprod Immunol"},{"key":"10.1016\/j.hoc.2025.02.003_bib86","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.jim.2014.04.006","article-title":"Validation of a multiplex electrochemiluminescent immunoassay platform in human and mouse samples","volume":"408","author":"Bastarache","year":"2014","journal-title":"J Immunol Methods"},{"issue":"5","key":"10.1016\/j.hoc.2025.02.003_bib87","doi-asserted-by":"crossref","first-page":"e281","DOI":"10.1097\/MPH.0000000000001791","article-title":"Hemophagocytic lymphohistiocytosis in children: clinical profile and outcome","volume":"42","author":"Simon","year":"2020","journal-title":"J Pediatr Hematol Oncol"},{"key":"10.1016\/j.hoc.2025.02.003_bib88","doi-asserted-by":"crossref","DOI":"10.3389\/fped.2023.1152409","article-title":"Presentations and outcomes of familial hemophagocytic lymphohistiocytosis in the pediatric intensive care units (PICUs)","volume":"11","author":"Alsohime","year":"2023","journal-title":"Front Pediatr"},{"issue":"17","key":"10.1016\/j.hoc.2025.02.003_bib89","doi-asserted-by":"crossref","first-page":"4577","DOI":"10.1182\/blood-2011-06-356261","article-title":"Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol","volume":"118","author":"Trottestam","year":"2011","journal-title":"Blood"},{"issue":"25","key":"10.1016\/j.hoc.2025.02.003_bib90","doi-asserted-by":"crossref","first-page":"2728","DOI":"10.1182\/blood-2017-06-788349","article-title":"Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study","volume":"130","author":"Bergsten","year":"2017","journal-title":"Blood"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib91","first-page":"e165","article-title":"Prognostic factors and long-term outcome in 52 Turkish children with hemophagocytic lymphohistiocytosis","volume":"16","author":"Kaya","year":"2015","journal-title":"Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensive Crit Care Soc"},{"key":"10.1016\/j.hoc.2025.02.003_bib92","doi-asserted-by":"crossref","DOI":"10.3389\/fped.2022.848004","article-title":"Epidemiology and outcomes of neonatal hemophagocytic lymphohistiocytosis","volume":"10","author":"Balakumar","year":"2022","journal-title":"Front Pediatr"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib93","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1080\/08880018.2020.1825575","article-title":"Clinical characteristics and outcomes of 101 children with hemophagocytic lymphohistiocytosis: a four-year single-center experience from Egypt","volume":"38","author":"Elsharkawy","year":"2021","journal-title":"Pediatr Hematol Oncol"},{"issue":"5","key":"10.1016\/j.hoc.2025.02.003_bib94","doi-asserted-by":"crossref","first-page":"1508","DOI":"10.1016\/j.jaip.2018.05.031","article-title":"Recommendations for the use of etoposide-based therapy and bone marrow transplantation for the treatment of HLH: Consensus statements by the HLH steering committee of the histiocyte society","volume":"6","author":"Ehl","year":"2018","journal-title":"J Allergy Clin Immunol Pract"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib95","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1007\/s12185-018-02572-z","article-title":"Outcomes in children with hemophagocytic lymphohistiocytosis treated using HLH-2004 protocol in Japan","volume":"109","author":"Yanagisawa","year":"2019","journal-title":"Int J Hematol"},{"issue":"10","key":"10.1016\/j.hoc.2025.02.003_bib96","doi-asserted-by":"crossref","first-page":"2255","DOI":"10.1007\/s00277-020-04209-w","article-title":"Treatment of pediatric primary hemophagocytic lymphohistiocytosis with the HLH-94\/2004 regimens and hematopoietic stem cell transplantation in China","volume":"99","author":"Ma","year":"2020","journal-title":"Ann Hematol"},{"issue":"10","key":"10.1016\/j.hoc.2025.02.003_bib97","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1182\/blood.2023022281","article-title":"Survival in primary hemophagocytic lymphohistiocytosis, 2016 to 2021: etoposide is better than its reputation","volume":"143","author":"B\u00f6hm","year":"2024","journal-title":"Blood"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib98","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1016\/j.bbmt.2018.01.022","article-title":"Outcomes of children with hemophagocytic lymphohistiocytosis given allogeneic hematopoietic stem cell transplantation in Italy","volume":"24","author":"Messina","year":"2018","journal-title":"Biol Blood Marrow Transplant J Am Soc Blood Marrow Transplant"},{"issue":"15","key":"10.1016\/j.hoc.2025.02.003_bib99","doi-asserted-by":"crossref","first-page":"3754","DOI":"10.1182\/bloodadvances.2020002101","article-title":"Stem cell transplantation for children with hemophagocytic lymphohistiocytosis: results from the HLH-2004 study","volume":"4","author":"Bergsten","year":"2020","journal-title":"Blood Adv"},{"issue":"19","key":"10.1016\/j.hoc.2025.02.003_bib100","doi-asserted-by":"crossref","first-page":"2088","DOI":"10.1182\/blood-2018-01-827485","article-title":"Treatment dilemmas in asymptomatic children with primary hemophagocytic lymphohistiocytosis","volume":"132","author":"Lucchini","year":"2018","journal-title":"Blood"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib101","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1182\/blood-2010-12-324533","article-title":"Perforin is a critical physiologic regulator of T-cell activation","volume":"118","author":"Lykens","year":"2011","journal-title":"Blood"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib102","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1046\/j.1365-2249.2003.02237.x","article-title":"Increased serum levels of interferon-gamma-inducible protein 10 and monokine induced by gamma interferon in patients with haemophagocytic lymphohistiocytosis","volume":"133","author":"Takada","year":"2003","journal-title":"Clin Exp Immunol"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib103","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1182\/blood-2003-10-3413","article-title":"An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder","volume":"104","author":"Jordan","year":"2004","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib104","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1002\/emmm.200900009","article-title":"Neutralization of IFNgamma defeats haemophagocytosis in LCMV-infected perforin- and Rab27a-deficient mice","volume":"1","author":"Pachlopnik Schmid","year":"2009","journal-title":"EMBO Mol Med"},{"issue":"19","key":"10.1016\/j.hoc.2025.02.003_bib105","doi-asserted-by":"crossref","first-page":"1811","DOI":"10.1056\/NEJMoa1911326","article-title":"Emapalumab in children with primary hemophagocytic lymphohistiocytosis","volume":"382","author":"Locatelli","year":"2020","journal-title":"N Engl J Med"},{"issue":"9","key":"10.1016\/j.hoc.2025.02.003_bib106","doi-asserted-by":"crossref","first-page":"2248","DOI":"10.1182\/bloodadvances.2023012217","article-title":"Real-world treatment patterns and outcomes in patients with primary hemophagocytic lymphohistiocytosis treated with emapalumab","volume":"8","author":"Chandrakasan","year":"2024","journal-title":"Blood Adv"},{"issue":"24","key":"10.1016\/j.hoc.2025.02.003_bib107","first-page":"01022","article-title":"JAK-STAT signaling pathway, immunodeficiency, inflammation, immune dysregulation, and inborn errors of immunity","volume":"S0091-6749","author":"Samra","year":"2024","journal-title":"J Allergy Clin Immunol"},{"issue":"13","key":"10.1016\/j.hoc.2025.02.003_bib108","doi-asserted-by":"crossref","first-page":"1666","DOI":"10.1182\/blood-2015-12-684399","article-title":"Janus kinase inhibition lessens inflammation and ameliorates disease in murine models of hemophagocytic lymphohistiocytosis","volume":"127","author":"Das","year":"2016","journal-title":"Blood"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib109","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1182\/blood-2016-02-700013","article-title":"Therapeutic effect of JAK1\/2 blockade on the manifestations of hemophagocytic lymphohistiocytosis in mice","volume":"128","author":"Maschalidi","year":"2016","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib110","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1182\/blood.2019000761","article-title":"Mechanisms of action of ruxolitinib in murine models of hemophagocytic lymphohistiocytosis","volume":"134","author":"Albeituni","year":"2019","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2025.02.003_bib111","doi-asserted-by":"crossref","DOI":"10.3389\/fphar.2021.650295","article-title":"Ruxolitinib, a JAK1\/2 inhibitor, ameliorates cytokine Storm in experimental models of hyperinflammation syndrome","volume":"12","author":"Huarte","year":"2021","journal-title":"Front Pharmacol"},{"key":"10.1016\/j.hoc.2025.02.003_bib112","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2023.1137037","article-title":"Cellular and transcriptional impacts of Janus kinase and\/or IFN-gamma inhibition in a mouse model of primary hemophagocytic lymphohistiocytosis","volume":"14","author":"Albeituni","year":"2023","journal-title":"Front Immunol"},{"issue":"23","key":"10.1016\/j.hoc.2025.02.003_bib113","doi-asserted-by":"crossref","first-page":"2386","DOI":"10.1182\/blood.2023021046","article-title":"Differential effects of itacitinib, fedratinib, and ruxolitinib in mouse models of hemophagocytic lymphohistiocytosis","volume":"143","author":"Keenan","year":"2024","journal-title":"Blood"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib114","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1182\/blood.2020006075","article-title":"JAK\/STAT pathway inhibition sensitizes CD8 T cells to dexamethasone-induced apoptosis in hyperinflammation","volume":"136","author":"Meyer","year":"2020","journal-title":"Blood"},{"issue":"24","key":"10.1016\/j.hoc.2025.02.003_bib115","doi-asserted-by":"crossref","first-page":"3493","DOI":"10.1182\/blood.2021014860","article-title":"A study of ruxolitinib response-based stratified treatment for pediatric hemophagocytic lymphohistiocytosis","volume":"139","author":"Zhang","year":"2022","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib116","doi-asserted-by":"crossref","first-page":"458","DOI":"10.3324\/haematol.2023.283478","article-title":"Ruxolitinib-based regimen in children with primary hemophagocytic lymphohistiocytosis","volume":"109","author":"Ge","year":"2024","journal-title":"Haematologica"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib117","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.28071","article-title":"Successful remission induction in refractory familial hemophagocytic lymphohistiocytosis with ruxolitinib as a bridge to hematopoietic stem cell transplantation","volume":"67","author":"Ramanan","year":"2020","journal-title":"Pediatr Blood Cancer"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib118","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.28954","article-title":"Successful management of familial hemophagocytic lymphohistiocytosis by the JAK 1\/2 inhibitor ruxolitinib","volume":"68","author":"Marois","year":"2021","journal-title":"Pediatr Blood Cancer"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib119","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1016\/j.immuni.2013.08.037","article-title":"Mechanisms of NOD-like receptor-associated inflammasome activation","volume":"39","author":"Wen","year":"2013","journal-title":"Immunity"},{"key":"10.1016\/j.hoc.2025.02.003_bib120","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1007\/978-3-031-59815-9_36","article-title":"IL-1 family blockade in cytokine Storm syndromes","volume":"1448","author":"Cron","year":"2024","journal-title":"Adv Exp Med Biol"},{"issue":"12","key":"10.1016\/j.hoc.2025.02.003_bib121","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.27997","article-title":"Anakinra as an agent to control hemophagocytic lymphohistiocytosis in Griscelli type 2","volume":"66","author":"Kim","year":"2019","journal-title":"Pediatr Blood Cancer"},{"issue":"6","key":"10.1016\/j.hoc.2025.02.003_bib122","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.28897","article-title":"Effect of COVID-19 on anakinra-induced remission in homozygous STX11 hemophagocytosis lymphohistiocytosis","volume":"68","author":"Vagrecha","year":"2021","journal-title":"Pediatr Blood Cancer"},{"key":"10.1016\/j.hoc.2025.02.003_bib123","doi-asserted-by":"crossref","DOI":"10.1038\/nrrheum.2018.11","article-title":"Autoinflammatory diseases: free IL-18 causes macrophage activation syndrome","author":"Lieben","year":"2018","journal-title":"Nat Rev Rheumatol"},{"key":"10.1016\/j.hoc.2025.02.003_bib124","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1007\/978-3-031-59815-9_40","article-title":"Other immunomodulatory treatment for cytokine Storm syndromes","volume":"1448","author":"Batu","year":"2024","journal-title":"Adv Exp Med Biol"},{"key":"10.1016\/j.hoc.2025.02.003_bib125","doi-asserted-by":"crossref","first-page":"239","DOI":"10.3389\/fimmu.2012.00239","article-title":"Protection from inflammatory organ damage in a murine model of hemophagocytic lymphohistiocytosis using treatment with IL-18 binding protein","volume":"3","author":"Chiossone","year":"2012","journal-title":"Front Immunol"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib126","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1002\/pbc.21019","article-title":"Etanercept for therapy-resistant macrophage activation syndrome","volume":"50","author":"Makay","year":"2008","journal-title":"Pediatr Blood Cancer"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib127","doi-asserted-by":"crossref","first-page":"137","DOI":"10.3109\/s10165-011-0477-9","article-title":"Adult-onset Still\u2019s disease with macrophage activation syndrome successfully treated with a combination of methotrexate and etanercept","volume":"22","author":"Maeshima","year":"2012","journal-title":"Mod Rheumatol"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib128","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1016\/j.cyto.2012.02.006","article-title":"Tocilizumab masks the clinical symptoms of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome: the diagnostic significance of interleukin-18 and interleukin-6","volume":"58","author":"Shimizu","year":"2012","journal-title":"Cytokine"},{"issue":"4","key":"10.1016\/j.hoc.2025.02.003_bib129","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1038\/mt.2014.242","article-title":"Perforin gene transfer into hematopoietic stem cells improves immune dysregulation in murine models of perforin deficiency","volume":"23","author":"Carmo","year":"2015","journal-title":"Mol Ther J Am Soc Gene Ther"},{"issue":"24","key":"10.1016\/j.hoc.2025.02.003_bib130","doi-asserted-by":"crossref","first-page":"2859","DOI":"10.1182\/blood-2016-07-729871","article-title":"Gene-corrected human Munc13-4-deficient CD8+ T cells can efficiently restrict EBV-driven lymphoproliferation in immunodeficient mice","volume":"128","author":"Soheili","year":"2016","journal-title":"Blood"},{"issue":"3","key":"10.1016\/j.hoc.2025.02.003_bib131","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1016\/j.jaci.2017.11.050","article-title":"T-cell gene therapy for perforin deficiency corrects cytotoxicity defects and prevents hemophagocytic lymphohistiocytosis manifestations","volume":"142","author":"Ghosh","year":"2018","journal-title":"J Allergy Clin Immunol"},{"issue":"8","key":"10.1016\/j.hoc.2025.02.003_bib132","doi-asserted-by":"crossref","first-page":"975","DOI":"10.1089\/hum.2019.025","article-title":"Retroviral UNC13D gene transfer restores cytotoxic activity of T cells derived from familial hemophagocytic lymphohistiocytosis type 3 patients in vitro","volume":"30","author":"Dettmer","year":"2019","journal-title":"Hum Gene Ther"},{"issue":"92","key":"10.1016\/j.hoc.2025.02.003_bib133","doi-asserted-by":"crossref","DOI":"10.1126\/sciimmunol.adi0042","article-title":"Precise CRISPR-Cas9 gene repair in autologous memory T cells to treat familial hemophagocytic lymphohistiocytosis","volume":"9","author":"Li","year":"2024","journal-title":"Sci Immunol"},{"issue":"25","key":"10.1016\/j.hoc.2025.02.003_bib134","doi-asserted-by":"crossref","first-page":"3281","DOI":"10.1182\/blood-2015-12-684498","article-title":"The minimum required level of donor chimerism in hereditary hemophagocytic lymphohistiocytosis","volume":"127","author":"Hartz","year":"2016","journal-title":"Blood"},{"issue":"27","key":"10.1016\/j.hoc.2025.02.003_bib135","doi-asserted-by":"crossref","first-page":"2781","DOI":"10.1182\/bloodadvances.2017012088","article-title":"Gene transfer into hematopoietic stem cells reduces HLH manifestations in a murine model of Munc13-4 deficiency","volume":"1","author":"Soheili","year":"2017","journal-title":"Blood Adv"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib136","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/j.jaci.2023.08.003","article-title":"Gene editing of hematopoietic stem cells restores T-cell response in familial hemophagocytic lymphohistiocytosis","volume":"153","author":"Dettmer-Monaco","year":"2024","journal-title":"J Allergy Clin Immunol"},{"issue":"23","key":"10.1016\/j.hoc.2025.02.003_bib137","doi-asserted-by":"crossref","first-page":"7258","DOI":"10.1182\/bloodadvances.2023010546","article-title":"Early response markers predict survival after etoposide-based therapy of hemophagocytic lymphohistiocytosis","volume":"7","author":"Verkamp","year":"2023","journal-title":"Blood Adv"},{"issue":"4","key":"10.1016\/j.hoc.2025.02.003_bib138","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1016\/j.jfma.2020.10.026","article-title":"Outcomes and prognostic factors associated with 180-day mortality in Taiwanese pediatric patients with Hemophagocytic Lymphohistiocytosis","volume":"120","author":"Yu","year":"2021","journal-title":"J Formos Med Assoc Taiwan Yi Zhi"},{"issue":"1","key":"10.1016\/j.hoc.2025.02.003_bib139","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1186\/s13023-022-02315-8","article-title":"Fulfillment status of hypertriglyceridemia and hypofibrinogenemia in children with hemophagocytic lymphohistiocytosis and risks of multiple organ dysfunction syndrome and early mortality","volume":"17","author":"Li","year":"2022","journal-title":"Orphanet J Rare Dis"},{"key":"10.1016\/j.hoc.2025.02.003_bib140","doi-asserted-by":"crossref","DOI":"10.3389\/fped.2022.941318","article-title":"Central nervous system involvement and thrombocytopenia as predictors of mortality in children with hemophagocytic lymphohistiocytosis","volume":"10","author":"Harnchoowong","year":"2022","journal-title":"Front Pediatr"},{"issue":"2","key":"10.1016\/j.hoc.2025.02.003_bib141","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1177\/0267659116667804","article-title":"Extracorporeal membrane oxygenation outcomes in children with hemophagocytic lymphohistiocytosis","volume":"32","author":"Cashen","year":"2017","journal-title":"Perfusion"},{"issue":"19","key":"10.1016\/j.hoc.2025.02.003_bib142","first-page":"2330","article-title":"A cell-based functional assay that accurately links genotype to phenotype in familial HLH","volume":"141","author":"Noori","year":"2023","journal-title":"Blood"}],"container-title":["Hematology\/Oncology Clinics of North America"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0889858825000188?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0889858825000188?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,5,24]],"date-time":"2025-05-24T01:20:37Z","timestamp":1748049637000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0889858825000188"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6]]},"references-count":143,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025,6]]}},"alternative-id":["S0889858825000188"],"URL":"https:\/\/doi.org\/10.1016\/j.hoc.2025.02.003","relation":{},"ISSN":["0889-8588"],"issn-type":[{"value":"0889-8588","type":"print"}],"subject":[],"published":{"date-parts":[[2025,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Clinical Characteristics and Treatment of Familial Hemophagocytic Lymphohistiocytosis","name":"articletitle","label":"Article Title"},{"value":"Hematology\/Oncology Clinics of North America","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.hoc.2025.02.003","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}]}}