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?
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.
Glucocerebroside
Sphingomyelin
Sulfatide
Ganglioside
Misc.