Familial lymphohistiocytosis is one of a group of familial diseases recently reported, probably related to Letterer-Siwe disease. These illnesses are almost invariably fatal in early childhood and are characterized pathologically by diffuse infiltration of most tissues by lymphocytes and large histiocytes. This may be the first reported case of extreme ocular involvement by this pathologic process.
Report of a Case
A 16-month-old boy with a three-month history of fever, vomiting, rash, and periorbital edema was admitted to Children's Hospital Medical Center. Previous examinations at another hospital showed hepatosplenomegaly, bizarre lymphocytes on bone marrow aspiration, and cirrhosis with round cell infiltration on liver biopsy. Although his fundi had been initially described as normal, he developed bilateral papilledema and what was thought to be uveitis in the right eye.The family history revealed a brother who died at 9 months of age at another hospital after a similar illness. Review of microscopic