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.

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.