{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T14:26:13Z","timestamp":1756995973049},"reference-count":86,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2013,1,16]],"date-time":"2013-01-16T00:00:00Z","timestamp":1358294400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Infect Dis"],"published-print":{"date-parts":[[2013,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Hemophagocytic syndrome (HPS) is clinically defined as a combination of fever, liver dysfunction, coagulation abnormalities, pancytopenia, progressive macrophage proliferation throughout the reticuloendothelial system, and cytokine over-production, and may be primary or secondary to infectious, auto-immune, and tumoral diseases. The most consistent association is with viral infections but, as it is still debated whether any micro-organisms are involved in its pathogenesis, we critically appraised the literature concerning HPS and its relationship with infections.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Infection-dependent HPS has been widely observed, but there are no data concerning its incidence in children. A better understanding of the pathophysiology of HPS may clarify the interactions between the immune system and the variously implicated potential infectious agents. Epstein-Barr virus (EBV) infection has been prominently associated with HPS, with clonal proliferation and the hyperactivation of EBV-infected T cells. However, a number of other viral, bacterial, fungal, and parasitic infections have been reported in association with HPS. In the case of low-risk HPS, corticosteroids and\/or intravenous immunoglobulin or cyclosporine A may be sufficient to control the biological process, but etoposide is recommended as a means of reversing infection-dependent lymphohistiocytic dysregulation in high-risk cases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Summary<\/jats:title><jats:p>HPS is a potential complication of various infections. A polymerase chain reaction search for infectious agents including EBV, cytomegalovirus and<jats:italic>Leishmania<\/jats:italic>is recommended in clinical settings characterised by non-remitting fever, organomegaly, cytopenia and hyperferritinemia.<\/jats:p><\/jats:sec>","DOI":"10.1186\/1471-2334-13-15","type":"journal-article","created":{"date-parts":[[2013,1,16]],"date-time":"2013-01-16T23:14:21Z","timestamp":1358378061000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Debate around infection-dependent hemophagocytic syndrome in paediatrics"],"prefix":"10.1186","volume":"13","author":[{"given":"Valentina","family":"Ansuini","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donato","family":"Rigante","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susanna","family":"Esposito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2013,1,16]]},"reference":[{"key":"2183_CR1","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1002\/rmv.638","volume":"20","author":"NR Maakaroun","year":"2010","unstructured":"Maakaroun NR, Moanna A, Jacob JT, Albrecht H: Viral infection associated with haemophagocytic syndrome. Rev Med Virol. 2010, 20: 93-105. 10.1002\/rmv.638.","journal-title":"Rev Med Virol"},{"key":"2183_CR2","doi-asserted-by":"publisher","first-page":"548","DOI":"10.1097\/00002281-200209000-00012","volume":"14","author":"A Ravelli","year":"2002","unstructured":"Ravelli A: Macrophage activation syndrome. Curr Opin Rheumatol. 2002, 14: 548-552. 10.1097\/00002281-200209000-00012.","journal-title":"Curr Opin Rheumatol"},{"key":"2183_CR3","first-page":"16","volume":"7","author":"E Douka","year":"2012","unstructured":"Douka E, Economidou F, Nanas S: Infections associated with the hemophagocytic syndrome. Hosp Chron. 2012, 7: 16-24.","journal-title":"Hosp Chron"},{"key":"2183_CR4","doi-asserted-by":"publisher","first-page":"58","DOI":"10.1532\/IJH97.07012","volume":"86","author":"E Ishii","year":"2007","unstructured":"Ishii E, Ohga S, Imashuku S, Yasukawa M, Tsuda H, Miura I, Yamamoto K, Horiuchi H, Takada K, Ohshima K, Nakamura S, Kinukawa N, Oshimi K, Kawa K: Nationwide survey of hemophagocytic lymphohistiocytosis in Japan. Int J Hematol. 2007, 86: 58-65. 10.1532\/IJH97.07012.","journal-title":"Int J Hematol"},{"key":"2183_CR5","doi-asserted-by":"publisher","first-page":"194","DOI":"10.1016\/S0140-6736(00)61951-7","volume":"2","author":"R Scott","year":"1939","unstructured":"Scott R, Robb-Smith A: Histiocytic medullary reticulosis. Lancet. 1939, 2: 194-198.","journal-title":"Lancet"},{"key":"2183_CR6","first-page":"29","volume":"18","author":"JI Henter","year":"1991","unstructured":"Henter JI, Elinder G, Ost A: Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society. Semin Oncol. 1991, 18: 29-33.","journal-title":"Semin Oncol"},{"key":"2183_CR7","doi-asserted-by":"publisher","first-page":"269","DOI":"10.1111\/j.1651-2227.1991.tb11849.x","volume":"80","author":"JI Henter","year":"1991","unstructured":"Henter JI, Elinder G: Familial hemophagocytic lymphohistiocytosis. Clinical review based on the findings in seven children. Acta Paediatr Scand. 1991, 80: 269-277. 10.1111\/j.1651-2227.1991.tb11849.x.","journal-title":"Acta Paediatr Scand"},{"key":"2183_CR8","first-page":"499","volume":"86","author":"M Allen","year":"2001","unstructured":"Allen M, De Fusco C, Legrand F, Clementi R, Conter V, Danesino C, Janka G, Aric\u00f2 M: Familial hemophagocytic lymphohistiocytosis: how late can the onset be?. Haematologica. 2001, 86: 499-503.","journal-title":"Haematologica"},{"key":"2183_CR9","doi-asserted-by":"publisher","first-page":"369","DOI":"10.1111\/j.1651-2227.1993.tb12699.x","volume":"82","author":"JI Henter","year":"1993","unstructured":"Henter JI, Ehrnst A, Andersson J, Elinder G: Familial hemophagocytic lymphohistiocytosis and viral infections. Acta Paediatr. 1993, 82: 369-372. 10.1111\/j.1651-2227.1993.tb12699.x.","journal-title":"Acta Paediatr"},{"key":"2183_CR10","doi-asserted-by":"publisher","first-page":"519","DOI":"10.1136\/adc.27.136.519","volume":"27","author":"J Farquhar","year":"1952","unstructured":"Farquhar J, Claireaux A: Familial haemophagocytic reticulosis. Arch Dis Child. 1952, 27: 519-525. 10.1136\/adc.27.136.519.","journal-title":"Arch Dis Child"},{"key":"2183_CR11","doi-asserted-by":"publisher","first-page":"428","DOI":"10.1111\/j.1651-2227.1991.tb11878.x","volume":"80","author":"JI Henter","year":"1991","unstructured":"Henter JI, Elinder G, Soder O, Ost A: Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand. 1991, 80: 428-435. 10.1111\/j.1651-2227.1991.tb11878.x.","journal-title":"Acta Paediatr Scand"},{"key":"2183_CR12","doi-asserted-by":"publisher","first-page":"276","DOI":"10.1002\/(SICI)1096-911X(199805)30:5<276::AID-MPO3>3.0.CO;2-C","volume":"30","author":"E Ishii","year":"1998","unstructured":"Ishii E, Ohga S, Tanimura M, Imashuku S, Sako M, Mizutani S, Miyazaki S: Clinical and epidemiologic studies of familial hemophagocytic lymphohistiocytosis in Japan. Japan LCH Study Group. Med Pediatr Oncol. 1998, 30: 276-283. 10.1002\/(SICI)1096-911X(199805)30:5<276::AID-MPO3>3.0.CO;2-C.","journal-title":"Med Pediatr Oncol"},{"key":"2183_CR13","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1080\/07853890500465189","volume":"38","author":"JW Verbsky","year":"2006","unstructured":"Verbsky JW, Grossman WJ: Hemophagocytic lymphohistiocytosis: diagnosis, pathophysiology, treatment, and future perspectives. Ann Med. 2006, 38: 20-31. 10.1080\/07853890500465189.","journal-title":"Ann Med"},{"key":"2183_CR14","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: 601-608. 10.3201\/eid0606.000608.","journal-title":"Emerg Infect Dis"},{"key":"2183_CR15","doi-asserted-by":"publisher","first-page":"767","DOI":"10.1007\/s00296-006-0280-7","volume":"27","author":"D Rigante","year":"2007","unstructured":"Rigante D, De Rosa G, Bertoni B, Ansuini V, Pardeo M, La Torraca I, Gaspari S, Stabile A: Large pericardial effusion requiring pericardiocentesis as cardinal sign of macrophage activation syndrome in systemic onset-juvenile idiopathic arthritis. Rheumatol Int. 2007, 27: 767-770. 10.1007\/s00296-006-0280-7.","journal-title":"Rheumatol Int"},{"key":"2183_CR16","doi-asserted-by":"publisher","first-page":"658","DOI":"10.1002\/art.22409","volume":"56","author":"D Rigante","year":"2007","unstructured":"Rigante D, Capoluongo E, Bertoni B, Ansuini V, Chiaretti A, Piastra M, Pulitan\u00f2 S, Genovese O, Compagnone A, Stabile A: First report of macrophage activation syndrome in hyperimmunoglobulinemia D with periodic fever syndrome. Arthritis Rheum. 2007, 56: 658-661. 10.1002\/art.22409.","journal-title":"Arthritis Rheum"},{"key":"2183_CR17","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1016\/S0889-8588(05)70521-9","volume":"12","author":"G Janka","year":"1998","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: 435-444. 10.1016\/S0889-8588(05)70521-9.","journal-title":"Hematol Oncol Clin North Am"},{"key":"2183_CR18","first-page":"142","volume":"1","author":"N Jabado","year":"2005","unstructured":"Jabado N, McCusker C, de Saint Basile G: Pediatric hemophagocytic syndromes: a diagnostic and therapeutic challenge. Allergy Asthma Clin Immunol. 2005, 1: 142-160.","journal-title":"Allergy Asthma Clin Immunol"},{"key":"2183_CR19","first-page":"53","volume":"10","author":"A Stabile","year":"2006","unstructured":"Stabile A, Bertoni B, Ansuini V, La Torraca I, Sall\u00ec A, Rigante D: The clinical spectrum and treatment options of macrophage activation syndrome in the pediatric age. Eur Rev Med Pharmacol Sci. 2006, 10: 53-59.","journal-title":"Eur Rev Med Pharmacol Sci"},{"key":"2183_CR20","doi-asserted-by":"crossref","first-page":"4100","DOI":"10.1182\/blood.V89.11.4100","volume":"89","author":"Y Osugi","year":"1997","unstructured":"Osugi Y, Hara J, Tagawa S, Takai K, Hosoi G, Matsuda Y, Ohta H, Fujisaki H, Kobayashi M, Sakata N, Kawa-Ha K, Okada S, Tawa A: Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis. Blood. 1997, 89: 4100-4103.","journal-title":"Blood"},{"key":"2183_CR21","doi-asserted-by":"crossref","first-page":"4706","DOI":"10.1182\/blood.V86.12.4706.bloodjournal86124706","volume":"86","author":"S Imashuku","year":"1995","unstructured":"Imashuku S, Hibi S, Sako M, Ishida Y, Mugishima H, Chen J, Tsunematsu Y: Soluble interleukin-2 receptor: a useful prognostic factor for patients with hemophagocytic lymphohistiocytosis. Blood. 1995, 86: 4706-4707.","journal-title":"Blood"},{"key":"2183_CR22","doi-asserted-by":"publisher","first-page":"814","DOI":"10.1016\/S1473-3099(07)70290-6","volume":"7","author":"NG Rouphael","year":"2007","unstructured":"Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C: Infections associated with haemophagocytic syndrome. Lancet Infect Dis. 2007, 7: 814-822. 10.1016\/S1473-3099(07)70290-6.","journal-title":"Lancet Infect Dis"},{"key":"2183_CR23","doi-asserted-by":"publisher","first-page":"993","DOI":"10.1002\/1097-0142(197909)44:3<993::AID-CNCR2820440329>3.0.CO;2-5","volume":"44","author":"RJ Risdall","year":"1979","unstructured":"Risdall RJ, McKenna RW, Nesbit ME, Krivit W, Balfour HH, Simmons RL, Brunning RD: Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer. 1979, 44: 993-1002. 10.1002\/1097-0142(197909)44:3<993::AID-CNCR2820440329>3.0.CO;2-5.","journal-title":"Cancer"},{"key":"2183_CR24","first-page":"157","volume":"37","author":"CJ Chen","year":"2004","unstructured":"Chen CJ, Huang YC, Jaing TH, Hung IJ, Yang CP, Chang LY, Lin TY: Hemophagocytic syndrome: a review of 18 pediatric cases. J Microbiol Immunol Infect. 2004, 37: 157-163.","journal-title":"J Microbiol Immunol Infect"},{"key":"2183_CR25","doi-asserted-by":"publisher","first-page":"373","DOI":"10.3109\/10428190009087028","volume":"38","author":"S Tabata YHibi","year":"2000","unstructured":"Tabata YHibi S, Teramura T, Kuriyama K, Yagi T, Todo S, Sawada T, Imashuku S: Molecular analysis of latent membrane protein 1 in patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in Japan. Leuk Lymphoma. 2000, 38: 373-380. 10.3109\/10428190009087028.","journal-title":"Leuk Lymphoma"},{"key":"2183_CR26","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1016\/S1040-8428(02)00117-8","volume":"44","author":"S Imashuku","year":"2002","unstructured":"Imashuku S: Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Crit Rev Oncol Hematol. 2002, 44: 259-272. 10.1016\/S1040-8428(02)00117-8.","journal-title":"Crit Rev Oncol Hematol"},{"key":"2183_CR27","doi-asserted-by":"publisher","first-page":"934","DOI":"10.1111\/j.1440-1827.1998.tb03864.x","volume":"48","author":"K Ohshima","year":"1998","unstructured":"Ohshima K, Suzumiya J, Sugihara M, Nagafuchi S, Ohga S, Kikuchi M: Clinicopathological study of severe chronic active Epstein-Barr virus infection that developed in association with lymphoproliferative disorder and\/or hemophagocytic syndrome. Pathol Int. 1998, 48: 934-943. 10.1111\/j.1440-1827.1998.tb03864.x.","journal-title":"Pathol Int"},{"key":"2183_CR28","doi-asserted-by":"publisher","first-page":"401","DOI":"10.3109\/10428199509112197","volume":"19","author":"IJ Su","year":"1995","unstructured":"Su IJ, Wang CH, Cheng AL, Chen RL: Hemophagocytic syndrome in Epstein-Barr virus-associated T lymphoproliferative disorders: disease spectrum, pathogenesis, and management. Leuk Lymphoma. 1995, 19: 401-406. 10.3109\/10428199509112197.","journal-title":"Leuk Lymphoma"},{"key":"2183_CR29","doi-asserted-by":"publisher","first-page":"1011","DOI":"10.1016\/j.ijporl.2005.02.014","volume":"69","author":"TL Lindemann","year":"2005","unstructured":"Lindemann TL, Greene JS: Persistent cervical lymphadenopathy in an adolescent with Epstein-Barr induced hemophagocytic syndrome: manifestations of a rare but often fatal disease. Int J Pediatr Otorhinolaryngol. 2005, 69: 1011-1014. 10.1016\/j.ijporl.2005.02.014.","journal-title":"Int J Pediatr Otorhinolaryngol"},{"key":"2183_CR30","doi-asserted-by":"publisher","first-page":"579","DOI":"10.1111\/j.1348-0421.2002.tb02737.x","volume":"46","author":"H Kimura","year":"2002","unstructured":"Kimura H, Hoshino Y, Hara S, Nishikawa K, Sako M, Hirayama M, Komada Y, Morishima T: Viral load in Epstein-Barr virus-associated hemophagocytic syndrome. Microbiol Immunol. 2002, 46: 579-582.","journal-title":"Microbiol Immunol"},{"key":"2183_CR31","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1080\/10428190210176","volume":"43","author":"T Teramura","year":"2002","unstructured":"Teramura T, Tabata Y, Yagi T, Morimoto A, Hibi S, Imashuku S: Quantitative analysis of cell-free Epstein-Barr virus genome copy number in patients with EBV-associated hemophagocytic lymphohistiocytosis. Leuk Lymphoma. 2002, 43: 173-179. 10.1080\/10428190210176.","journal-title":"Leuk Lymphoma"},{"key":"2183_CR32","first-page":"572","volume":"82","author":"JS Chen","year":"1997","unstructured":"Chen JS, Tzeng CC, Tsao CJ, Su WC, Chen TY, Jung YC, Su IJ: Clonal karyotype abnormalities in EBV associated hemophagocytic syndrome. Haematologica. 1997, 82: 572-576.","journal-title":"Haematologica"},{"key":"2183_CR33","doi-asserted-by":"publisher","first-page":"1969","DOI":"10.1172\/JCI119728","volume":"100","author":"JD Lay","year":"1997","unstructured":"Lay JD, Tsao CJ, Chen JY, Kadin ME, Su IJ: Upregulation of tumor necrosis factor-alpha gene by Epstein-Barr virus and activation of macrophages in Epstein-Barr virus-infected T cells in the pathogenesis of hemophagocytic syndrome. J Clin Invest. 1997, 100: 1969-1979. 10.1172\/JCI119728.","journal-title":"J Clin Invest"},{"key":"2183_CR34","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1093\/ajcp\/97.2.189","volume":"97","author":"F Craig","year":"1991","unstructured":"Craig F, Clare N, Sklar J, Banks P: T-cell lymphoma and the virus-associated hemophagocytic syndrome. Am J Clin Pathol. 1991, 97: 189-194.","journal-title":"Am J Clin Pathol"},{"key":"2183_CR35","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1002\/(SICI)1096-911X(199809)31:3<131::AID-MPO1>3.0.CO;2-C","volume":"31","author":"S Imashuku","year":"1998","unstructured":"Imashuku S, Hibi S, Tabata Y, Sako M, Sekine Y, Hirayama K, Sakazaki H, Maeda N, Kito H, Shichino H, Mugishima H: Biomarker and morphological characteristics of Epstein-Barr virus-related hemophagocy lymphohistiocytosis. Med Pediatr Oncol. 1998, 31: 131-137. 10.1002\/(SICI)1096-911X(199809)31:3<131::AID-MPO1>3.0.CO;2-C.","journal-title":"Med Pediatr Oncol"},{"key":"2183_CR36","doi-asserted-by":"publisher","first-page":"1437","DOI":"10.1517\/14656566.2.9.1437","volume":"2","author":"S Imashuku","year":"2001","unstructured":"Imashuku S, Teramura T, Morimoto A, Hibi S: Recent developments in the management of haemophagocytic lymphohistiocytosis. Expert Opin Pharmacother. 2001, 2: 1437-1448. 10.1517\/14656566.2.9.1437.","journal-title":"Expert Opin Pharmacother"},{"key":"2183_CR37","first-page":"1253","volume":"42","author":"S Bakhshi","year":"2005","unstructured":"Bakhshi S, Pautu JL: EBV associated hemophagocytic lymphohistiocytosis with spontaneous regression. Indian Pediatr. 2005, 42: 1253-1255.","journal-title":"Indian Pediatr"},{"key":"2183_CR38","doi-asserted-by":"publisher","first-page":"37","DOI":"10.3109\/10428190009053537","volume":"39","author":"S Imashuku","year":"2000","unstructured":"Imashuku S, Tabata Y, Teramura T, Hibi S: Treatment strategies for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH). Leuk Lymphoma. 2000, 39: 37-49. 10.3109\/10428190009053537.","journal-title":"Leuk Lymphoma"},{"key":"2183_CR39","doi-asserted-by":"publisher","first-page":"569","DOI":"10.1097\/MPH.0b013e3180f61be3","volume":"29","author":"NJ Balamuth","year":"2007","unstructured":"Balamuth NJ, Nichols KE, Paessler M, Teachey DT: Use of rituximab in conjunction with immunosuppressive chemotherapy as a novel therapy for Epstein Barr virus-associated hemophagocytic lymphohistiocytosis. J Pediatr Hematol Oncol. 2007, 29: 569-573. 10.1097\/MPH.0b013e3180f61be3.","journal-title":"J Pediatr Hematol Oncol"},{"key":"2183_CR40","doi-asserted-by":"publisher","first-page":"569","DOI":"10.1038\/sj.bmt.1701620","volume":"23","author":"S Imashuku","year":"1999","unstructured":"Imashuku S, Hibi S, Todo S, Sako M, Inoue M, Kawa K, Koike K, Iwai A, Tsuchiya S, Akiyama Y, Kotani T, Kawamura Y, Hirosawa M, Hasegawa D, Kosaka Y, Yamaguchi H, Ishii E, Kato K, Ishii M, Kigasawa H: Allogeneic hematopoietic stem cell transplantation for patients with hemophagocytic syndrome (HPS) in Japan. Bone Marrow Transplant. 1999, 23: 569-572. 10.1038\/sj.bmt.1701620.","journal-title":"Bone Marrow Transplant"},{"key":"2183_CR41","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1542\/peds.75.2.280","volume":"75","author":"EH Danish","year":"1985","unstructured":"Danish EH, Dahms BB, Kumar ML: Cytomegalovirus-associated hemophagocytic syndrome. Pediatrics. 1985, 75: 280-283.","journal-title":"Pediatrics"},{"key":"2183_CR42","doi-asserted-by":"publisher","first-page":"368","DOI":"10.1007\/s12185-009-0261-1","volume":"89","author":"A Abdelkefi","year":"2009","unstructured":"Abdelkefi A, Ben Jamil W, Torjman L, Ladeb S, Ksouri H, Lakhal A, Ben Hassen A, Ben Abdeladhim A, Ben Othman T: Hemophagocytic syndrome after hematopoietic stem cell transplantation: a prospective observational study. Int J Hematol. 2009, 89: 368-373. 10.1007\/s12185-009-0261-1.","journal-title":"Int J Hematol"},{"key":"2183_CR43","doi-asserted-by":"publisher","first-page":"315","DOI":"10.1007\/s00431-005-1636-9","volume":"164","author":"S Imashuku","year":"2005","unstructured":"Imashuku S, Ueda I, Teramura T, Mori K, Morimoto A, Sako M, Ishii E: Occurrence of haemophagocytic lymphohistiocytosis at less than 1 year of age: analysis of 96 patients. Eur J Pediatr. 2005, 164: 315-319. 10.1007\/s00431-005-1636-9.","journal-title":"Eur J Pediatr"},{"key":"2183_CR44","first-page":"100","volume":"23","author":"M Amenomori","year":"2005","unstructured":"Amenomori M, Migita K, Miyashita T, Yoshida S, Ito M, Eguchi K, Ezaki H: Cytomegalovirus-associated hemophagocytic syndrome in a patient with adult onset Still\u2019s disease. Clin Exp Rheumatol. 2005, 23: 100-102.","journal-title":"Clin Exp Rheumatol"},{"key":"2183_CR45","doi-asserted-by":"publisher","first-page":"1112","DOI":"10.1046\/j.1365-2141.2000.02478.x","volume":"111","author":"RD Pastore","year":"2000","unstructured":"Pastore RD, Chadburn A, Kripas C, Schattner EJ: Novel association of haemophagocytic syndrome with Kaposi\u2019s sarcoma-associated herpesvirus-related primary effusion lymphoma. Br J Haematol. 2000, 111: 1112-1115. 10.1046\/j.1365-2141.2000.02478.x.","journal-title":"Br J Haematol"},{"key":"2183_CR46","doi-asserted-by":"publisher","first-page":"285","DOI":"10.1086\/375224","volume":"37","author":"L Fardet","year":"2003","unstructured":"Fardet L, Blum L, Kerob D, Agbalika F, Galicier L, Dupuy A, Lafaurie M, Meignin V, Morel P, Lebb\u00e9 C: Human herpesvirus 8-associated hemophagocytic lymphohistiocytosis in human immunodeficiency virus-infected patients. Clin Infect Dis. 2003, 37: 285-291. 10.1086\/375224.","journal-title":"Clin Infect Dis"},{"key":"2183_CR47","doi-asserted-by":"publisher","first-page":"1203","DOI":"10.1182\/blood-2005-03-0950","volume":"106","author":"WJ Grossman","year":"2005","unstructured":"Grossman WJ, Radhi M, Schauer D, Gerday E, Grose C, Goldman FD: Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8. Blood. 2005, 106: 1203-1206. 10.1182\/blood-2005-03-0950.","journal-title":"Blood"},{"key":"2183_CR48","doi-asserted-by":"publisher","first-page":"361","DOI":"10.1111\/j.1600-0609.2007.00828.x","volume":"78","author":"A Re","year":"2007","unstructured":"Re A, Facchetti F, Borlenghi E, Cattaneo C, Capucci MA, Ungari M, Barozzi P, Vallerini D, Potenza L, Torelli G, Rossi G, Luppi M: Fatal hemophagocytic syndrome related to active human herpesvirus-8\/Kaposi sarcoma-associated herpesvirus infection in human immunodeficiency virusnegative, non-transplant patients without related malignancies. Eur J Haematol. 2007, 78: 361-364. 10.1111\/j.1600-0609.2007.00828.x.","journal-title":"Eur J Haematol"},{"key":"2183_CR49","doi-asserted-by":"publisher","first-page":"855","DOI":"10.1016\/S0248-8663(97)81959-5","volume":"18","author":"L Sailler","year":"1997","unstructured":"Sailler L, Duchayne E, Marchou B, Brousset P, Pris J, Massip P, Corberand J, Arlet P: Etiological aspects of reactive hemophagocytoses: retrospective study in 99 patients. Rev Med Interne. 1997, 18: 855-864. 10.1016\/S0248-8663(97)81959-5.","journal-title":"Rev Med Interne"},{"key":"2183_CR50","first-page":"853","volume":"121","author":"H Albrecht","year":"1997","unstructured":"Albrecht H, Schafer H, Stellbrink HJ, Greten H: Epstein-Barr virus\u2013Associated hemophagocytic syndrome. A cause of fever of unknown origin in human immunodeficiency virus infection. Arch Pathol Lab Med. 1997, 121: 853-858.","journal-title":"Arch Pathol Lab Med"},{"key":"2183_CR51","doi-asserted-by":"publisher","first-page":"1539","DOI":"10.1097\/01.qai.0000136725.92886.b8","volume":"37","author":"HY Sun","year":"2004","unstructured":"Sun HY, Chen MY, Fang CT, Hsieh SM, Hung CC, Chang SC: Hemophagocytic lymphohistiocytosis: an unusual initial presentation of acute HIV infection. J Acquir Immune Defic Syndr. 2004, 37: 1539-1540. 10.1097\/01.qai.0000136725.92886.b8.","journal-title":"J Acquir Immune Defic Syndr"},{"key":"2183_CR52","doi-asserted-by":"publisher","first-page":"e161","DOI":"10.1086\/379615","volume":"37","author":"S Bhatia","year":"2003","unstructured":"Bhatia S, Bauer F, Bilgrami SA: Candidiasis associated hemophagocytic lymphohistiocytosis in a patient infected with human immunodeficiency virus. Clin Infect Dis. 2003, 37: e161-e166. 10.1086\/379615.","journal-title":"Clin Infect Dis"},{"key":"2183_CR53","doi-asserted-by":"publisher","first-page":"231","DOI":"10.3109\/10428190109097696","volume":"42","author":"MV Preciado","year":"2001","unstructured":"Preciado MV, De Matteo E, Fallo A, Chabay P, Drelichman G, Grinstein S: EBV-associated Hodgkin\u2019s disease in an HIV-infected child presenting with a hemophagocytic syndrome. Leuk Lymphoma. 2001, 42: 231-234. 10.3109\/10428190109097696.","journal-title":"Leuk Lymphoma"},{"key":"2183_CR54","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1136\/jcp.44.4.297","volume":"44","author":"MN Potter","year":"1991","unstructured":"Potter MN, Foot AB, Oakhill A: Influenza A and the virus-associated haemophagocytic syndrome: cluster of three cases in children with acute leukaemia. J Clin Pathol. 1991, 44: 297-299. 10.1136\/jcp.44.4.297.","journal-title":"J Clin Pathol"},{"key":"2183_CR55","doi-asserted-by":"publisher","first-page":"1183","DOI":"10.2169\/internalmedicine.45.1736","volume":"45","author":"M Ando","year":"2006","unstructured":"Ando M, Miyazaki E, Hiroshige S, Ashihara Y, Okubo T, Ueo M, Fukami T, Sugisaki K, Tsuda T, Ohishi K, Yoshitake S, Noguchi T, Kumamoto T: Virus associated hemophagocytic syndrome accompanied by acute respiratory failure caused by influenza A (H3N2). Intern Med. 2006, 45: 1183-1186. 10.2169\/internalmedicine.45.1736.","journal-title":"Intern Med"},{"key":"2183_CR56","doi-asserted-by":"publisher","first-page":"799","DOI":"10.1007\/s00431-003-1288-6","volume":"162","author":"T Watanabe","year":"2003","unstructured":"Watanabe T, Okazaki E, Shibuya H: Influenza A virus-associated encephalopathy with haemophagocytic syndrome. Eur J Pediatr. 2003, 162: 799-800. 10.1007\/s00431-003-1288-6.","journal-title":"Eur J Pediatr"},{"key":"2183_CR57","doi-asserted-by":"publisher","first-page":"e216","DOI":"10.1542\/peds.2005-1861","volume":"118","author":"SS Mou","year":"2006","unstructured":"Mou SS, Nakagawa TA, Riemer EC, McLean TW, Hines MH, Shetty AK: Hemophagocytic lymphohistiocytosis complicating influenza A infection. Pediatrics. 2006, 118: e216-e219. 10.1542\/peds.2005-1861.","journal-title":"Pediatrics"},{"key":"2183_CR58","doi-asserted-by":"crossref","first-page":"446","DOI":"10.7883\/yoken.JJID.2008.446","volume":"61","author":"R Samransamruajkit","year":"2008","unstructured":"Samransamruajkit R, Hiranrat T, Chieochansin T, Sritippayawan S, Deerojanawong J, Prapphal N, Poovorawan Y: Prevalence, clinical presentations and complications among hospitalized children with influenza pneumonia. Jpn J Infect Dis. 2008, 61: 446-449.","journal-title":"Jpn J Infect Dis"},{"key":"2183_CR59","doi-asserted-by":"publisher","first-page":"735","DOI":"10.1016\/j.humpath.2008.08.015","volume":"40","author":"Z Zhang","year":"2009","unstructured":"Zhang Z, Zhang J, Huang K, Li KS, Yuen KY, Guan Y, Chen H, Ng WF: Systemic infection of avian influenza A virus H5N1 subtype in humans. Hum Pathol. 2009, 40: 735-739. 10.1016\/j.humpath.2008.08.015.","journal-title":"Hum Pathol"},{"key":"2183_CR60","doi-asserted-by":"publisher","first-page":"243","DOI":"10.4065\/73.3.243","volume":"73","author":"K Kimura","year":"1998","unstructured":"Kimura K, Adlakha A, Simon PM: Fatal case of swine influenza virus in an immunocompetent host. Mayo Clin Proc. 1998, 73: 243-245. 10.4065\/73.3.243.","journal-title":"Mayo Clin Proc"},{"key":"2183_CR61","doi-asserted-by":"publisher","first-page":"1831","DOI":"10.1016\/S0140-6736(02)11772-7","volume":"360","author":"CY Cheung","year":"2002","unstructured":"Cheung CY, Poon LL, Lau AS, Luk W, Lau YL, Shortridge KF, Gordon S, Guan Y, Peiris JS: Induction of proinflammatory cytokines in human macrophages by influenza A (H5N1) viruses: a mechanism for the unusual severity of human disease?. Lancet. 2002, 360: 1831-1837. 10.1016\/S0140-6736(02)11772-7.","journal-title":"Lancet"},{"key":"2183_CR62","doi-asserted-by":"publisher","first-page":"4530","DOI":"10.4049\/jimmunol.176.8.4530","volume":"176","author":"SM Hsieh","year":"2006","unstructured":"Hsieh SM, Chang SC: Insufficient perforin expression in CD8\u00fe T cells in response to hemagglutinin from avian influenza (H5N1) virus. J Immunol. 2006, 176: 4530-4533.","journal-title":"J Immunol"},{"key":"2183_CR63","doi-asserted-by":"publisher","first-page":"870","DOI":"10.1016\/S0140-6736(06)68232-9","volume":"367","author":"JI Henter","year":"2006","unstructured":"Henter JI, Chow CB, Leung CW, Lau YL: Cytotoxic therapy for severe avian influenza A (H5N1) infection. Lancet. 2006, 367: 870-873. 10.1016\/S0140-6736(06)68232-9.","journal-title":"Lancet"},{"key":"2183_CR64","doi-asserted-by":"crossref","first-page":"149","DOI":"10.7883\/yoken.JJID.2005.149","volume":"58","author":"Z Kaya","year":"2005","unstructured":"Kaya Z, Ozturk G, Gursel T, Bozdayi G: Spontaneous resolution of hemophagocytic syndrome and disseminated intravascular coagulation associated with Parvovirus b19 infection in a previously healthy child. Jpn J Infect Dis. 2005, 58: 149-151.","journal-title":"Jpn J Infect Dis"},{"key":"2183_CR65","doi-asserted-by":"publisher","first-page":"8610","DOI":"10.1002\/pbc.20807","volume":"47","author":"S Yilmaz","year":"2006","unstructured":"Yilmaz S, Oren H, Demircioglu F, Firinci F, Korkmaz A, Irken G: Parvovirus B19: a cause for aplastic crisis and hemophagocytic lymphohistiocytosis. Pediatr Blood Canc. 2006, 47: 8610-","journal-title":"Pediatr Blood Canc"},{"key":"2183_CR66","doi-asserted-by":"publisher","first-page":"739","DOI":"10.1007\/s10096-002-0793-2","volume":"21","author":"C Larroche","year":"2002","unstructured":"Larroche C, Scieux C, Honderlick P, Piette AM, Morinet F, Bletry O: Spontaneous resolution of hemophagocytic syndrome associated with acute Parvovirus B19 infection and concomitant Epstein-Barr virus reactivation in an otherwise healthy adult. Eur J Clin Microbiol Infect Dis. 2002, 21: 739-742. 10.1007\/s10096-002-0793-2.","journal-title":"Eur J Clin Microbiol Infect Dis"},{"key":"2183_CR67","first-page":"118","volume":"24","author":"U Dutta","year":"2005","unstructured":"Dutta U, Mittal S, Ratho RK, Das A: Acute liver failure and severe hemophagocytosis secondary to Parvovirus B19 infection. Indian J Gastroenterol. 2005, 24: 118-119.","journal-title":"Indian J Gastroenterol"},{"key":"2183_CR68","doi-asserted-by":"publisher","first-page":"1340","DOI":"10.1111\/j.1600-6143.2008.02244.x","volume":"8","author":"MR Ardalan","year":"2008","unstructured":"Ardalan MR, Shoja MM, Tubbs RS, Esmaili H, Keyvani H: Postrenal transplant hemophagocytic lymphohistiocytosis and thrombotic microangiopathy associated with Parvovirus B19 infection. Am J Transplant. 2008, 8: 1340-1344. 10.1111\/j.1600-6143.2008.02244.x.","journal-title":"Am J Transplant"},{"key":"2183_CR69","doi-asserted-by":"publisher","first-page":"1188","DOI":"10.2169\/internalmedicine.41.1188","volume":"41","author":"M Watanabe","year":"2002","unstructured":"Watanabe M, Shibuya A, Okuno J, Maeda T, Tamama S, Saigenji K: Hepatitis A virus infection associated with hemophagocytic syndrome: report of two cases. Intern Med. 2002, 41: 1188-1192. 10.2169\/internalmedicine.41.1188.","journal-title":"Intern Med"},{"key":"2183_CR70","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1590\/S0036-46652008000200012","volume":"50","author":"FF Tuon","year":"2008","unstructured":"Tuon FF, Gomes VS, Amato VS, Graf ME, Fonseca GH, Lazari C, Nicodemo AC: Hemophagocytic syndrome associated with hepatitis A: case report and literature review. Rev Inst Med Trop Sao Paulo. 2008, 50: 123-127.","journal-title":"Rev Inst Med Trop Sao Paulo"},{"key":"2183_CR71","first-page":"166","volume":"35","author":"RA Russo","year":"2008","unstructured":"Russo RA, Rosenzweig SD, Katsicas MM: Hepatitis A-associated macrophage activation syndrome in children with systemic juvenile idiopathic arthritis: report of 2 cases. J Rheumatol. 2008, 35: 166-168.","journal-title":"J Rheumatol"},{"key":"2183_CR72","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1007\/s00431-007-0436-9","volume":"167","author":"K Katsibardi","year":"2008","unstructured":"Katsibardi K, Moschovi MA, Theodoridou M, Spanakis N, Kalabalikis P, Tsakris A, Tzortzatou-Stathopoulou F: Enterovirus-associated hemophagocytic syndrome in children with malignancy: report of three cases and review of the literature. Eur J Pediatr. 2008, 167: 97-102.","journal-title":"Eur J Pediatr"},{"key":"2183_CR73","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1146\/annurev-med-041610-134208","volume":"63","author":"GE Janka","year":"2012","unstructured":"Janka GE: Familial and acquired hemophagocytic lymphohistiocytosis. Annu Rev Med. 2012, 63: 233-46. 10.1146\/annurev-med-041610-134208.","journal-title":"Annu Rev Med"},{"key":"2183_CR74","doi-asserted-by":"publisher","first-page":"447","DOI":"10.1016\/S1473-3099(06)70524-2","volume":"6","author":"PK Brastianos","year":"2006","unstructured":"Brastianos PK, Swanson JW, Torbenson M, Sperati J, Karakousis PC: Tuberculosis-associated haemophagocytic syndrome. Lancet Infect Dis. 2006, 6: 447-454. 10.1016\/S1473-3099(06)70524-2.","journal-title":"Lancet Infect Dis"},{"key":"2183_CR75","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1111\/j.1525-1470.1996.tb00723.x","volume":"13","author":"A Rositto","year":"1996","unstructured":"Rositto A, Molinaro L, Larralde M, Ranalletta M, Drut R: Disseminated cutaneous eruption after BCG vaccination. Pediatr Dermatol. 1996, 13: 451-454. 10.1111\/j.1525-1470.1996.tb00723.x.","journal-title":"Pediatr Dermatol"},{"key":"2183_CR76","doi-asserted-by":"publisher","first-page":"334","DOI":"10.1002\/ajh.21098","volume":"83","author":"I Sari","year":"2008","unstructured":"Sari I, Altuntas F, Hacioglu S, Kocyigit I, Sevinc A, Sacar S, Deniz K, Alp E, Eser B, Yildiz O, Kaynar L, Unal A, Cetin M: A multicenter retrospective study defining the clinical and haematological manifestations of brucellosis and pancytopenia in a large series: hematological malignancies, the unusual cause of pancytopenia in patients with brucellosis. Am J Hematol. 2008, 83: 334-339. 10.1002\/ajh.21098.","journal-title":"Am J Hematol"},{"key":"2183_CR77","doi-asserted-by":"publisher","first-page":"181","DOI":"10.1556\/AMicr.57.2010.3.3","volume":"57","author":"HH Niller","year":"2010","unstructured":"Niller HH: Myelodysplastic syndrome (MDS) as a late stage of subclinical hemophagocytic lymphohistiocytosis (HLH): a putative role for Leptospira infection. A hypothesis. Acta Microbiol Immunol Hung. 2010, 57: 181-189. 10.1556\/AMicr.57.2010.3.3.","journal-title":"Acta Microbiol Immunol Hung"},{"issue":"Pt 4","key":"2183_CR78","doi-asserted-by":"publisher","first-page":"537","DOI":"10.1099\/jmm.0.025833-0","volume":"60","author":"A Cascio","year":"2011","unstructured":"Cascio A, Giordano S, Dones P, Venezia S, Iaria C, Ziino O: Haemophagocytic syndrome and rickettsial diseases. J Med Microbiol. 2011, 60 (Pt 4): 537-542.","journal-title":"J Med Microbiol"},{"key":"2183_CR79","first-page":"223","volume":"24","author":"JB Gosh","year":"2009","unstructured":"Gosh JB, Roy M, Bala A: Infection associated with hemophagocytic lymphohistiocytosis triggered by nosocomial infection. Oman Med J. 2009, 24: 223-225.","journal-title":"Oman Med J"},{"key":"2183_CR80","first-page":"1324","volume":"16","author":"A Cascio","year":"2012","unstructured":"Cascio A, Pernice LM, Barberi G, Delfino D, Biondo C, Beninati C, Mancuso G, Rodriguez-Morales AJ, Iaria C: Secondary hemophagocytic lymphohistiocytosis in zoonoses. A systematic review. Eur Rev Med Pharmacol Sci. 2012, 16: 1324-1337.","journal-title":"Eur Rev Med Pharmacol Sci"},{"key":"2183_CR81","doi-asserted-by":"publisher","first-page":"E58","DOI":"10.1542\/peds.106.4.e58","volume":"106","author":"MH Gagnaire","year":"2000","unstructured":"Gagnaire MH, Galambrun C, Stephan JL: Hemophagocytic syndrome: a misleading complication of visceral leishmaniasis in children - A series of 12 cases. Pediatrics. 2000, 106: E58-10.1542\/peds.106.4.e58.","journal-title":"Pediatrics"},{"key":"2183_CR82","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1111\/j.1365-2257.2005.00717.x","volume":"27","author":"ZN Singh","year":"2005","unstructured":"Singh ZN, Rakheja D, Yadav TP, Shome DK: Infection-associated haemophagocytosis: the tropical spectrum. Clin Lab Haematol. 2005, 27: 312-315. 10.1111\/j.1365-2257.2005.00717.x.","journal-title":"Clin Lab Haematol"},{"key":"2183_CR83","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1016\/S0163-4453(98)93594-0","volume":"36","author":"K Numata","year":"1998","unstructured":"Numata K, Tsutsumi H, Wakai S, Tachi N, Chiba S: A child case of haemophagocytic syndrome associated with cryptococcal meningoencephalitis. J Infect. 1998, 36: 118-119. 10.1016\/S0163-4453(98)93594-0.","journal-title":"J Infect"},{"key":"2183_CR84","doi-asserted-by":"publisher","first-page":"1463","DOI":"10.1093\/clinids\/21.6.1463","volume":"21","author":"PR Koduri","year":"1995","unstructured":"Koduri PR, Chundi V, DeMarais P, Mizock BA, Patel AR, Weinstein RA: Reactive hemophagocytic syndrome: a new presentation of disseminated histoplasmosis in patients with AIDS. Clin Infect Dis. 1995, 21: 1463-1465. 10.1093\/clinids\/21.6.1463.","journal-title":"Clin Infect Dis"},{"key":"2183_CR85","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/j.jinf.2008.11.010","volume":"58","author":"M De Lavaissiere","year":"2009","unstructured":"De Lavaissiere M, Manceron V, Bour\u00e9e P, Gar\u00e7on L, Bisaro F, Delfraissy JF, Lambotte O, Goujard C: Reconstitution inflammatory syndrome related to histoplasmosis, with a hemophagocytic syndrome in HIV infection. J Infect. 2009, 58: 245-247. 10.1016\/j.jinf.2008.11.010.","journal-title":"J Infect"},{"key":"2183_CR86","first-page":"426","volume":"84","author":"K Chokephaibulkit","year":"2001","unstructured":"Chokephaibulkit K, Veerakul G, Vanprapar N, Chaiprasert A, Tanphaichitr V, Chearskul S: Penicilliosis-associated hemophagocytic syndrome in a human immunodeficiency virus-infected child: the first case report in children. J Med Assoc Thai. 2001, 84: 426-429.","journal-title":"J Med Assoc Thai"}],"container-title":["BMC Infectious Diseases"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/1471-2334-13-15.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/1471-2334-13-15\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/1471-2334-13-15","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/1471-2334-13-15.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,4]],"date-time":"2024-05-04T20:02:31Z","timestamp":1714852951000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcinfectdis.biomedcentral.com\/articles\/10.1186\/1471-2334-13-15"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,1,16]]},"references-count":86,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2013,12]]}},"alternative-id":["2183"],"URL":"https:\/\/doi.org\/10.1186\/1471-2334-13-15","relation":{},"ISSN":["1471-2334"],"issn-type":[{"value":"1471-2334","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,1,16]]},"assertion":[{"value":"1 January 2013","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 January 2013","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 January 2013","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"15"}}