Ocular and cerebral involvement in familial lymphohistiocytosis.

A 5-month-old infant girl died of familial lymphohistiocytosis. The central nervous system showed widespread perivascular infiltration of the cerebral pia and arachnoid, the cerebral white matter and deep gray matter, the cerebellum, and brain stem by lymphocytes, benign appearing histiocytes, and macrophages with erythro-and lymphophagocytosis. The eyes had mild infiltration of the anterior uveal tract, moderate involvement of the inner retina, and marked infiltration of the optic nerves by identical cells.