Gaucher Liver cells

Fig. 31.15 Gaucher s disease. Gaucher cells (sphingolipid-storing macrophages) within the liver parenchyma (arrow), shown here as pale-blue cells with an internal structure similar to cigarette paper (PAS)...

In the adult form of Gaucher s disease, glucosylcerebrosides accumulate in liver, spleen, and bone-marrow cells. Although the common galactosylceramides and their derivatives are found in the tissues of affected patients, accumulations of galactosylcerebrosides or their metabolites are not found, nor do ceramides accumulate. What enzyme activity is probably deficient in patients with Gaucher s disease ... 

The common identity of human acid ]0-D-glucosidase and j3-D-xylosidase is indicated by similar binding patterns of the glycoside hydrolase activities of each enzyme to various lectins, by similar ratios between their intra- and extracellular levels in normal and I-cell fibroblasts, and by their deficiencies in liver tissues from patients with Gaucher disease, ... 

Cerebrosides accumulate in the reticuloendothelial cells in Gaucher s disease. The cerebroside content of spleen and liver is increased tenfold however, an increase in the cerebroside content of brain has not been convincingly demonstrated. 

In contrast to what happens in Gaucher s disease, the accretion of lipoid material in the liver does not induce fibrous proliferation. Although spleen and liver are the primary targets of the disease, the lipoid material sometimes accumulates in alveolar cells of the lung and in the endothelial cells of the small capillaries of the lung. These lesions appear on a radiological picture as mottling or miliary nodules. Careful exa-... 

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