OCULAR INVOLVEMENT AND VISUAL SEQUELAE IN HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN ASSOCIATION WITH CONGENITAL DYSFIBRINOGENEMIA

We report a 7-year-old girl with hemophagocytic lymphohistiocytosis an d an uncommon complication of vascular retinopathy and visual sequelae . She was also verified to be complicated with dysfibrogenemia. The pa tient was treated with etoposide, dexamethasone, and cyclosporin A. Du ring the fifth hospital week, a generalized tonic-clonic convulsion de veloped followed by deep coma. When she regained full consciousness, s he complained of a visual disturbance. The ophthalmologic examination showed bilateral extensive retinal edema with numerous cotton-wool spo ts, indicative of vaso-occlusive retinopathy. This is inconsistent wit h the main finding of precious cases with ocular involvement, namely p apilledema. Severely reduced visual acuity and visual field defects re mained in both eyes even after systemic therapy. An awareness of this rare but serious complication is important because it may be preventab le by early institution of the chemotherapy. The pathogenesis of the r etinopathy is also discussed.