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-yearold 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 EpsteinBarr 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.