Retinal Necrosis in X-linked Lymphoproliferative Disease

• Published in: Ophthalmology (Rochester, MN) Vol. 101; no. 4; pp. 705 - 709
• Main Authors: Grossniklaus, Hans E., Aaberg, Thomas M., Purnell, Edward W., Luka, Janos, Seemayer, Thomas A.
• Format: Journal Article Conference Proceeding
• Language: English
• Published: New York, NY Elsevier Inc 01.04.1994; Elsevier
• Subjects: Biological and medical sciences; Child; DNA, Viral - analysis; Eye Infections, Viral - complications; Eye Infections, Viral - pathology; Hematologic and hematopoietic diseases; Herpesvirus 4, Human - genetics; Humans; Infectious Mononucleosis - complications; Infectious Mononucleosis - pathology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoproliferative Disorders - complications; Lymphoproliferative Disorders - pathology; Male; Medical sciences; Retinal Necrosis Syndrome, Acute - complications; Retinal Necrosis Syndrome, Acute - microbiology; Retinal Necrosis Syndrome, Acute - pathology; Visual Acuity; Gammaherpesvirinae; Human; Sex linked character; Retinopathy; Immune response; Pathogenesis; Herpesviridae; Retina; Malignant hemopathy; Epstein Barr virus; Necrosis; Virus; Case study; Eye disease; Lymphoproliferative syndrome; Complication; Child
• ISSN: 0161-6420; 1549-4713
• DOI: 10.1016/S0161-6420(94)31275-9

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.