title: Catastrophic hemophagocytic lymphohistiocytosis in a young man with nephrotic syndrome.
abstract: BACKGROUND
  In its early stage, necrotizing fasciitis mimics a milder cutaneous infection, such as cellulitis, and is an uncommon but potentially fatal complication of nephrotic syndrome. It may trigger an uncontrolled and catastrophic immune response, such as hemophagocytic lymphohistiocytosis (HLH).
  
  
  METHODS
  A 19-y-old man presented with steroid-resistant nephrotic syndrome and rapidly progressing Escherichia coli monomicrobial necrotizing fasciitis with bacteremia. The conditions developed one day after steroid therapy, leading to multiple organ dysfunction syndrome. A provisional diagnosis of HLH was promptly made, based upon the patient's fever, unremitting shock, marked pancytopenia, hyperferritinemia, hypofibrinogenemia, and the typical histiocytic hemophagocytosis in pleural effusion. Despite aggressive medical treatment and organ support, the patient died 8 days after transfer to our intensive care unit. Final bone marrow examination confirmed the diagnosis of HLH.
  
  
  CONCLUSIONS
  Although nephrotic syndrome associated with E. coli infection is common, this is the first reported case of E. coli monomicrobial necrotizing fasciitis with bacteremia resulting in HLH in a patient with nephrotic syndrome.
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan.
authors.forename: Chin-Chun
authors.initials: CC
authors.lastname: Chang
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan; Division of Nephrology, Department of Internal Medicine, Taoyuan Armed Forces General Hospital, Taoyuan, Taiwan.
authors.forename: Po-Jen
authors.initials: PJ
authors.lastname: Hsiao
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Taoyuan Armed Forces General Hospital, Taoyuan, Taiwan; Division of Infectious Disease, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan.
authors.forename: Chih-Chien
authors.initials: CC
authors.lastname: Chiu
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan.
authors.forename: Ying-Chieh
authors.initials: YC
authors.lastname: Chen
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan.
authors.forename: Shih-Hua
authors.initials: SH
authors.lastname: Lin
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan.
authors.forename: Chia-Chao
authors.initials: CC
authors.lastname: Wu
authors.affiliation: Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan. Electronic address: dgschen@ndmctsgh.edu.tw.
authors.forename: Jin-Shuen
authors.initials: JS
authors.lastname: Chen
mesh_terms: D000328:Adult; D002388:Catastrophic Illness; D004927:Escherichia coli Infections / Q000150:complications* / Q000175:diagnosis; D017809:Fatal Outcome; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis; D008297:Male; D009404:Nephrotic Syndrome / Q000150:complications* / Q000175:diagnosis; D055815:Young Adult
keywords: Hemophagocytic lymphohistiocytosis; Necrotizing fasciitis; Nephrotic syndrome