The Submicroscopic Morphology of Gaucher Cells

Abstract

Abstract The large cells characteristically found in the bone marrow and other organs in Gaucher’s disease have been reinvestigated with the electron microscope, as well as with phase contrast and polarizing microscopes and by standard staining methods. The cytoplasm was filled with a number of dense elongate or crescent shaped bodies. Each of these fibrils was in turn seen with the electron microscope to be bounded by a single dense limiting membrane, and to contain a homogeneous appearing matrix in which were embedded numerous tubular-appearing subunits. These tubular elements measured approximately 130 Å in diameter, and were of very great length. It is suggested that these submicroscopic tubular structures could represent the molecular kerasin or lipoprotein units known from biochemical and histochemical evidence to be present in Gaucher cells. Some details about the appearance of the ectoplasm in spread cells, and the area near the cell border in cells fixed in situ, are reported. Electron micrographs revealed a complex cell border which was extended into many small pseudopodia and ridge-like projections. Numerous microvesicles about 600 Å in diameter were found in the area near the cell membrane. This is taken as possible evidence of transport of some substance across an active cell membrane. An attempt is made to correlate these morphologic findings with the clinical and biochemical findings of ourselves and others on Gaucher’s disease.