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Retinal necrosis in X-linked lymphoproliferative disease
H E Grossniklaus1, T M Aaberg, E W Purnell
1Department of Ophthalmology, Emory University, Atlanta, GA.
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.
Conclusion:
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.
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