title: Survival rates and prognostic factors of Epstein-Barr virus-associated hydroa vacciniforme and hypersensitivity to mosquito bites.
abstract: BACKGROUND
  Epstein-Barr virus (EBV)-associated T/natural-killer lymphoproliferative disorders form a group of diseases that includes classical and systemic hydroa vacciniforme (HV) and hypersensitivity to mosquito bites (HMB). Patients with systemic HV (sHV) and HMB often have a poor prognosis, although little is known about the prognostic factors.
  
  
  OBJECTIVE
  To elucidate the prognostic factors of HV and HMB.
  
  
  METHODS
  We studied clinicopathological manifestations, routine laboratory findings, anti-EBV titres, EBV DNA load and EBV-encoded gene expression, including expression of BZLF1, in 50 patients with classical HV (cHV), sHV, HMB only and HMB with HV (HMB + HV), and further analysed 30 patients who were available for follow-up.
  
  
  RESULTS
  The median age of disease onset was 5 years (range 1-74). A follow-up study indicated that fatal outcomes were observed in three of eight patients with sHV, two of six patients with HMB only, and two of five patients with HMB + HV. The main causes of death were complications from haematopoietic stem-cell transplantation and multiorgan failure. There were no fatalities among the 11 patients with cHV. Univariate analysis revealed two poor prognostic indicators: (i) onset age > 9 years and (ii) the expression of an EBV-encoded immediate-early gene transcript, BZLF1 mRNA, in the skin lesions (P < 0·001 and P = 0·003, respectively).
  
  
  CONCLUSIONS
  No prognostic correlation was observed in EBV-infected lymphocyte subsets, anti-EBV antibody titres or EBV DNA load. Late onset and EBV reactivation are both related to more severe phenotypes of the disease, and thus may predict a poor prognosis.
authors.affiliation: Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama, 700-8558, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Miyake
authors.forename: T
authors.initials: T
authors.lastname: Yamamoto
authors.forename: Y
authors.initials: Y
authors.lastname: Hirai
authors.forename: M
authors.initials: M
authors.lastname: Otsuka
authors.forename: T
authors.initials: T
authors.lastname: Hamada
authors.forename: K
authors.initials: K
authors.lastname: Tsuji
authors.forename: S
authors.initials: S
authors.lastname: Morizane
authors.forename: D
authors.initials: D
authors.lastname: Suzuki
authors.forename: Y
authors.initials: Y
authors.lastname: Aoyama
authors.forename: K
authors.initials: K
authors.lastname: Iwatsuki
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000818:Animals; D002648:Child; D002675:Child, Preschool; D009033:Culicidae; D020031:Epstein-Barr Virus Infections / Q000401:mortality*; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D006837:Hydroa Vacciniforme / Q000401:mortality* / Q000821:virology; D006967:Hypersensitivity / Q000401:mortality* / Q000821:virology; D054019:Immune Reconstitution Inflammatory Syndrome / Q000821:virology; D007223:Infant; D007299:Insect Bites and Stings / Q000401:mortality* / Q000821:virology; D053208:Kaplan-Meier Estimate; D007963:Leukocytes, Mononuclear / Q000821:virology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D055815:Young Adult