title: Treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in young adults: a report from the HLH study center.
abstract: BACKGROUND
  Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), also known as EBV-associated hemophagocytic syndrome, develops mostly in children and young adults and may be fatal. Early etoposide treatment has been confirmed to be effective in children. However, it is unclear whether the same treatment is useful in adults.
  
  
  METHODS
  To assess whether etoposide is effective in treating young adult cases, we retrospectively studied the therapeutic measures taken and outcomes in 20 young adult cases of EBV-HLH. Eleven cases were registered in our HLH study center in Kyoto and nine derived from the literature. The patients were between 17 and 33 years old and eight were males. The influence of gender, cell lineage (T- or natural killer-), EBV serology pattern, jaundice and treatment on the outcome was assessed.
  
  
  CONCLUSIONS
  Patients receiving etoposide within four weeks after diagnosis had a good prognosis as five of the seven patients survived compared to one of 13 not treated with etoposide or treated late (chi-square test for survival, P = 0.0095). The Kaplan-Meier analysis showed the 2.5-year survival of 85.7 +/- 13.2% in the early etoposide-treated patients, compared to 10.3 +/- 9.4% in the remaining patients (log-rank test, P = 0.0141). Thus, early etoposide treatment is effective in treating EBV-HLH in young adults as well as in children.
authors.affiliation: Kyoto City Institute of Health and Environmental Sciences, Kyoto, Japan. shinim95@mbox.kyoto-inet.or.jp
authors.forename: Shinsaku
authors.initials: S
authors.lastname: Imashuku
authors.forename: Kikuko
authors.initials: K
authors.lastname: Kuriyama
authors.forename: Rika
authors.initials: R
authors.lastname: Sakai
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Nakao
authors.forename: Shin-ichi
authors.initials: S
authors.lastname: Masuda
authors.forename: Norimasa
authors.initials: N
authors.lastname: Yasuda
authors.forename: Fumio
authors.initials: F
authors.lastname: Kawano
authors.forename: Kimikazu
authors.initials: K
authors.lastname: Yakushijin
authors.forename: Akiko
authors.initials: A
authors.lastname: Miyagawa
authors.forename: Taisei
authors.initials: T
authors.lastname: Nakao
authors.forename: Tomoko
authors.initials: T
authors.lastname: Teramura
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Tabata
authors.forename: Akira
authors.initials: A
authors.lastname: Morimoto
authors.forename: Shigeyoshi
authors.initials: S
authors.lastname: Hibi
mesh_terms: D000293:Adolescent; D000328:Adult; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use*; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005047:Etoposide / Q000627:therapeutic use*; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000972:Antineoplastic Agents, Phytogenic; D005047:Etoposide