Retinal necrosis in X-linked lymphoproliferative disease.
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.
Grossniklaus
H E
HE
Department of Ophthalmology, Emory University, Atlanta, GA.
Aaberg
T M
TM
Purnell
E W
EW
Luka
J
J
Seemayer
T A
TA
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
D004279:DNA, Viral