Gaucher disease

 

 

This autosomal recessive entity results from mutations in the gene encoding glucocerebrosidase. In the low and high magnification photos above the darker staining cells are surviving hepatocytes. The lighter areas consist of Kupffer cells distended with accumulated glucocerebrosides. Such accumulations are found also in tissue macrophages in spleen, lymph nodes, and bone marrow also.

 

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