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April 1964

Retinal Involvement With Reticuloendotheliosis of Unclassified Type

Author Affiliations

Howe Laboratory of Ophthalmology, Harvard Medical School, Massachusetts Eye and Ear Infirmary; Department of Medicine, Massachusetts General Hospital.

Arch Ophthalmol. 1964;71(4):489-491. doi:10.1001/archopht.1964.00970010505009

The reticuloendothelioses (lipidoses) characteristically associated with retinal involvement are Tay-Sachs disease, Niemann-Pick disease, and metachromatic leukoencephalopathy. The patient to be reported in this paper had clinical signs suggestive of the adult form of Gaucher's disease and pathochemical signs suggestive of Niemann-Pick disease. The abnormalities in the fundi are thought to be unlike those reported in any other form of reticuloendotheliosis.

Report of Case  The patient was a 24-year-old woman, who had been known to have hepatosplenomegaly since childhood. She had been diagnosed at the age of 12 years as having Gaucher's disease. The present hospital admission was for consideration of splenectomy to prevent complications of pregnancy.At the age of 6 she had bled so profusely at the time of a tonsillectomy that she was hospitalized for two weeks and (probably) had a transfusion. At the age of 12 the large liver and spleen were noted and a diagnosis was

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