Page 272 - Haematologica Atlas of Hematologic Cytology

Figure 5 iemann iemann Pic Pic disease disease Bone marrow with hematopoie c c c c c c cells cells and foamy cells cells sca ered or grouped together Niemann-Pick disease disease is is is is a a a a a a a a a a a a a a a sphingomyelin sphingomyelin lipido- sis with large lipid- lled macrophages due to to de ciency of acid sphingomyelinase AB
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Figure 6 iemann Pic disease Bone marrow smear ( and ) Large fat-laden macrophages with abundant cytoplasm and and a a a a a a a a a a small small nucleus They di er from normal fat cells as the sphingomyelin droplets are small small and and rela vely uniform in in in size ( ( ) ) Sea-blue his ocytes may be observed (D) The typical foamy cells stain stain weakly with Sudan black stain stain for lipids 259