title: Debate around infection-dependent hemophagocytic syndrome in paediatrics.
abstract: BACKGROUND
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  HPS is a potential complication of various infections. A polymerase chain reaction search for infectious agents including EBV, cytomegalovirus and Leishmania is recommended in clinical settings characterised by non-remitting fever, organomegaly, cytopenia and hyperferritinemia.
authors: Pediatric Clinic 1, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122, Milan, Italy.
authors: Valentina
authors: V
authors: Ansuini
authors: Donato
authors: D
authors: Rigante
authors: Susanna
authors: S
authors: Esposito
mesh_terms: D000293:Adolescent; D001424:Bacterial Infections / Q000150:complications; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007239:Infections / Q000150:complications*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*; D009181:Mycoses / Q000150:complications; D010272:Parasitic Diseases / Q000150:complications; D010372:Pediatrics; D014777:Virus Diseases / Q000150:complications