Renal involvement in adult Gaucher's disease after splenectomy.

Gaucher's disease with severe clinical and pathologic renal involvement is exceptionally rare. This article describes severe renal involvement and proteinuria in a young black woman with Gaucher's disease. The accumulation of glucocerebroside (Gaucher bodies) in glomerular mesangial and endothelial cells and in interstitial cells of the kidney is indicative of the phagocytic potential and the deficiency of glucocerebroside-cleaving enzyme in these cells. Severe proteinuria in this patient was attributed to the extensive glomerular involvement by Gaucher's disease.