Page 273 - Haematologica Atlas of Hematologic Cytology

CHAPTER 33 - Storage diseases
AB
CD
EF
Figure 7 Sea blue blue blue blue his his his ocytosis Bone marrow smear from a a a a a a a a a a a a a woman presen ng with with with splenomegaly and and thrombocyto- penia ( and and ) Numerous sea-blue his his his his ocytes ocytes i i i i i i i i i i i i i i i i i i i e e e e e e e e e e e e e e e e e e e e e e e e e e e e e e e e his his his his ocytes ocytes lled with with with granules that stain stain blue blue blue blue blue or or or blue-green with with with May-Gr nwald Giemsa or or or or other other panop c c c c c c c c c c c stains Sea-blue his his his ocytosis is is is is is is is a a a a a a a a a a a a a a a a a a a a a a a a a rare inherited lysosomal storage disease It is is is is is is is is is characterized by accumula on on on of of of of of ceroid or or or or lipofuscin in in in in in the the the macrophages of of of of of bone marrow liver spleen and other other organs Sea-blue Sea-blue his his ocytosis is is is is is is considered a a a a a a a a a a a a a a a a a a a a a a a a variant of of of of of Niemann-Pick disease as as as as reduced levels of of of of of cell sphingomye- linase have been reported in in in in in in in in some cases Sea-blue Sea-blue his his ocytes are are are brown in in in in in in in in unstained lms they are are are periodic acid acid Schi posi ve ve ve ( ( ( ( ( ( ) ) ) ) ) and and are are are stained stained by Ziehl-Nielsen (acid fast) stain stain stain stain (D) Nile blue blue sulphate stain stain stain stain for for phospholipids ( ( ( ( ( ( ) ) ) ) ) and and Oil red O O for for lipids lipids (F (F le le ) ) ) ) ) ) Moreover under a a a a a a a a a a a a a a a a a a a uorescence uorescence microscope they show strong auto uorescence uorescence (F (F right) It should be be noted that an an an increase in in in in bone marrow blue-sea his ocytes can occasionally be be observed in in in in other condi ons such as as as as as as as as myeloprolifera ve ve neoplasms immune thrombocytopenia hemoglobinopathies hyperlipopro- teinemia chronic granulomatous disease as as as as as as well as as as as as as in in in in in in in other lipid lipid storage disorders The increase in in in in in in in cellular cellular cataboli- sm and excessive accumula on on of intracellular lipids could play an an an an important pathogene c c c c c c c c c c c c role in in in in in in the the acquired forms 260