The ultrastructure of vacuolated lymphocytes in the peripheral blood of juvenile amaurotic familial idiocy.

Abstract

Diagnostic value of vacuolated lymphocytes in the peripheral blood of juvenile amaurotic familial idiocy (AFI) was emphasized. Vacuoles were never stained with Sudan III, Sudan black B and Nile blue cytochemically. The abnormal corpuscles were observed by the electron microscope in the cells identified as lymphocytes. The vacuoles in Giemsa stained smears may correspond to these abnormal granules. Round bodies were the commonest, with an outer smooth limiting membrane, surrounding an inner vacant zone partly filled with electron-dense, amorphous or parallel double membrane-like materials. It was noteworthy that there was another kind of unique bodies which were composed of two or three concentrically arranged layers. The structure of these unusual bodies may be substantially the same as that of one of the membranous cytoplasmic bodies (MCB) found by Terry and Weiss. In conclusion, it seems likely that the vacuoles within lymphocytes contain lipid. If it can be asserted that the MCB-like bodies are exactly the same as MCB, the relation-or lack of it-between vacuolated lymphocytes of juvenile AFI and the fundamental metabolic disturbance of the disease will be made clear.