title: Retinal necrosis in X-linked lymphoproliferative disease.
abstract: BACKGROUND
  X-linked lymphoproliferative disease is a hereditary disease that renders the males susceptible to fatal infectious mononucleosis, aplastic anemia, malignant lymphoma, and hypogammaglobulinemia after infection by the Epstein-Barr virus.
  
  
  METHODS
  The authors examined the clinical and pathologic findings in an 8-year-old boy with bilateral retinal necrosis who died with aplastic anemia as a complication of X-linked lymphoproliferative disease.
  
  
  RESULTS
  Results of histopathologic examination of the eyes disclosed retinal necrosis, and examination using the polymerase chain reaction technique showed Epstein-Barr virus genomic DNA in the left eye.
  
  
  CONCLUSIONS
  Retinal necrosis may be part of the expanding spectrum of X-linked lymphoproliferative disease. It is unknown if the retinal necrosis is due directly to Epstein-Barr virus infection or a host inflammatory response.
authors.affiliation: Department of Ophthalmology, Emory University, Atlanta, GA.
authors.forename: H E
authors.initials: HE
authors.lastname: Grossniklaus
authors.forename: T M
authors.initials: TM
authors.lastname: Aaberg
authors.forename: E W
authors.initials: EW
authors.lastname: Purnell
authors.forename: J
authors.initials: J
authors.lastname: Luka
authors.forename: T A
authors.initials: TA
authors.lastname: Seemayer
mesh_terms: D002648:Child; D004279:DNA, Viral / Q000032:analysis; D015828:Eye Infections, Viral / Q000150:complications / Q000473:pathology; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D007244:Infectious Mononucleosis / Q000150:complications / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000473:pathology; D008297:Male; D015882:Retinal Necrosis Syndrome, Acute / Q000150:complications* / Q000382:microbiology / Q000473:pathology; D014792:Visual Acuity
chemical_list: D004279:DNA, Viral