Back to Homepage

Case 186 - Discussion

Hematopathology Case

 

Niemann-Pick Disease

  • Sphingomyelinase deficiency
  • Sphingomyelin accumulates in macrophages and neurons
  • Organs most severely affected: brain, spleen, liver, bone marrow, lymph nodes, lungs
  • Morphology: foamy macrophages, not the "winkled paper" type in Gaucher disease
  • EM: “Zebra body storage”