A 48 year old lady presented with hepatosplenomegaly with pancytopenia. On microscopic examination of bone marrow cells, crumpled tissue paper appearance is seen. Which is the product likely to have accumulated?: (A) Glucocerebroside, (B) Sphingomyelin, (C) Sulfatide, (D) Ganglioside
Answer is A. Crumpled tissue paper appearance of cytoplasm + organomegaly and pancytopenia suggests storage disorder Lysosomal storage of glucocerebroside (Gaucher disease) in cells of the monocyte-macrophage system leads to a characteristic cellular alteration of these cells Gaucher cells- present in the bone marrow, spleen, lymph nodes, hepatic sinusoids, and other organs and tissues in all forms of GD.