Surface marker and other characteristics of Gaucher's cells

Abstract

The histiocytic nature of Gaucher's cells has been inferred from their phagocytic ability, high lysosomal enzyme content, and distinctively reticuloendothelial distribution. However, this view has not been universally accepted and, in addition, immunologic mechanisms have been invoked in the pathogenesis of the disease. Immunologic surface marker studies, which have proved of great value in the characterisation of the pathologic cells of a wide variety of other hematologic proliferations, should therefore contribute to further understanding of the nature of the pathognomonic cells of Gaucher's disease. No previous surface-marker study of Gaucher's disease has been described, and in the present paper a full surface marker study of the splenic storage cells in a girl of 8 with Gaucher's disease is reported. It largely substantiates the monocyte/histiocyte nature of Gaucher's cells. In addition, an apparent T-lymphocyte deficiency is demonstrated in the spleen (splenectomy) and peripheral blood (electronmicroscopy). The possible significance of this finding is discussed.