<prompt> Answer this question truthfullyWhat lysosomal storage disease is identified by the presence of a cherry red spot on the macula and hepatosplenomegaly?  <response>: <reason> Niemann-Pick disease is the lysosomal storage disease that is characterized by a cherry red spot on the macula and hepatosplenomegaly. Niemann-Pick disease is caused by a deficiency of the enzymes acid sphingomyelinase types A and B or sphingomyelin phosphodiesterase 1 type C, which leads to the accumulation of sphingomyelin in the bodys cells and tissues, including the liver, spleen, and brain. The buildup of sphingomyelin can cause enlargement of the liver and spleen hepatosplenomegaly and a cherry red spot to appear on the macula, which is the center of the retina. The presence of a cherry red spot on the macula and hepatosplenomegaly can help to confirm a diagnosis of Niemann-Pick disease. 