The morphogenesis of Gaucher cells investigated by electron microscopy.

Recently this problem has been investigated by studying the submicroscopic morphology of Gaucher cells in the bone marrow,”2 spleen3 and liver.4’5 These studies have demonstrated that the cerebroside or the cerebrosideprotein complex ( Uzman’s lipoprotein6) is aggregated in tubular elements which appear as dense rings in cross section, the outside diameter measuring about 400 A. Avariable amount of tubules, together with a pale matrix, is contained in cytoplasmic vacuoles, called Gaucher bodies. These very characteristic bodies, because of their high content of acid phosphatase activity and other enzymes, could be considered lysosomal in nature.3 Moreover, Lee et al.7 have found that isolated cerebroside has the same tubular morphology as observed in the Gaucher cells. Nevertheless, the precise mechanism involved in the accumulation of intracellular cerebroside has not vet been completely elucidated. De Marsh and Kautz’ support the hypothesis that the Gaucher cells incorporate a lipoprotein precursor of extracellular origin by pinocytosis rather than by endogenous synthesis. On the contrary, Ross et al.8 and Fisher and Reidbord3 have found some morphologic features of the Gaucher bodies which suggest that the mitochondria participate in the formation of these structures. Lee et al.,7 studying iron metabolism in Gaucher’s disease, support the concept that destroyed phagocytized red cells are the source of cerebroside in the Gaucher cells. Finally, support for a lack of glucocerebroside-cleaving enzyme comes from studies by Brady et al.#{176} using glucose-114C cerebroside as substrate. The results of Fraccaro et al.1#{176} with in vitro culture of spleen cells from a case of Gaucher’s disease are in keeping with the hypothesis of cerebroside accumulation by phagocytic activity. The present paper is concerned with the fine structure of the Gaucher cell in the spleen and liver. In particular, we have investigated the possible correlation between erythrophagocytic activity and the morphogenesis of Gaucher bodies.