- 22.Pediatric pathology
- (7)Congenital metabolic disease (Gaucher disease)
Microscopic view, high power, (H&E); In the absence of beta-glucocerebrosidase, glucocerebroside cannot be catalytically converted into ceramide and glucose and thus it accumulated in histiocytes (lysosome).These histiocytes take on a large vacuolated appearance and are termed Gaucher cells. These are seen in bone marrow, liver, spleen and lymph node with striated cytoplasm (often described as resembling wrinkled tissue paper)
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