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SJ McGonegle, VS Hershberger, RK Hutchins, S Schneider, DP Witte, R Harris; EBV-Related Bilateral Acute Retinal Necrosis in a Patient With X-Linked Lymphoproliferative Disease (XLPD) . Invest. Ophthalmol. Vis. Sci. 2002;43(13):4327.
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Purpose: The present case discusses a rare EBV-positive retinal biopsy in a patient with X-linked lymphoproliferative disease. XLPD is a spectrum of conditions including fatal infectious mononucleosis, hypo/agammaglobulinemia, B-cell lymphomas in extranodal sites, and aplastic anemia associated with a deficient immune response to EBV. To our knowledge there is only one report in the literature of retinal necrosis associated with XLPD. We present fundus photographs and pathologic findings in a patient with EBV-related acute retinal necrosis. Methods: A 10-month-old boy with XLPD was referred for bilateral retinal infiltrates. Examination of the right eye revealed an afferent pupillary defect, posterior synechiae, rubeosis, vitreous haze, yellow-white opacification of the entire retina with multiple retinal hemorrhages, and nasal retinal detachment. Left eye examination revealed 3 small vitreous fluff balls, pronounced white swelling and hemorrhaging of the optic nerve head, whitish edema of the retina with retinal hemorrhages, and widespread patchy RPE atrophy and scarring. Diagnostic vitrectomy and retinal biopsy were performed on the right eye, and evaluated for the presence of viral DNA, toxoplasma gondii, and bacterial and fungal cultures using in situ hybridization techniques. Results: Fundus photographs documented a white retinal infiltrate along with multiple depigmented oval-shaped lesions measuring approximately 300u in diameter at the level of the retinal pigment epithelium. Retinal biopsy showed hemorrhagic and necrotic tissue interspersed with atypical lymphocytes. In situ hybridization studies of the retinal biopsy were positive for EBV, while tests for other viruses, bacteria, and fungi were negative. Conclusion: This paper lends support to the rare association between XLPD and EBV with retinal necrosis. However, the paucity of culture-proven EBV in ocular lesions and the lack of an animal model renders it difficult to establish a direct causal role for the virus in retinal necrosis.
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