Splenic macrophages in Gaucher's disease differ from those in Ceroid Histiocytosis by staining positive for

a)lipids
b)phospholipids
c)acid fast stain
d)iron
Ans & Ref.
Iron

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Robbins ch.5 fig 5.16

The glucocerebrosides accumulate in massive amounts within phagocytic cells throughout the body in all forms of Gaucher disease. The distended phagocytic cells, known as Gaucher cells, are found in the spleen, liver, bone marrow, lymph nodes, tonsils, thymus, and Peyer patches. Similar cells may be found in both the alveolar septa and the air spaces in the lung. In contrast to the lipid storage diseases already discussed, Gaucher cells rarely appear vacuolated but instead have a fibrillary type of cytoplasm likened to crumpled tissue paper ( Fig. 5-16 ). Gaucher cells are often enlarged, sometimes up to 100 µm in diameter, and have one or more dark, eccentrically placed nuclei. Periodic acid-Schiff (PAS) staining is usually intensely positive. With the electron microscope, the fibrillary cytoplasm can be resolved as elongated, distended lysosomes, containing the stored lipid in stacks of bilayers.[29]

Figure 5-16 Gaucher disease involving the bone marrow. A, Gaucher cells with abundant lipid-laden granular cytoplasm. B, Electron micrograph of Gaucher cells with elongated distended lysosomes. (Courtesy of Dr. Matthew Fries, Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX.)
Lipid Storage Diseases : PathCONSULT

Spleen

http://www.medscape.com/viewarticle/410718_3

The histochemical staining properties of ceroid histiocytosis have been extensively studied.[7-13] In summary, Wright's and Giemsa staining imparts a sea-blue color, which also accounts for the descriptive term SBH. Positivity is observed by a PAS reaction both before and after diastase digestion; by autofluorescence and acid-fast fluorescence, with reactions tending to increase with aging of the slide; and with Sudan black stain. Ceroid does not react with Kinyoun carbolfuchsin or Prussian blue stains or in using Fite's method.
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Gaucher’s disease

Positive stains: iron, PAS (but weak)

Negative stains: phospholipids stains, acid-fast stains

DD: chronic myelogenous leukemia (similar looking cells-PseudoGaucher Cells)
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Lipid histiocytoses

Includes ceroid (sea-blue) histiocytosis, Gaucher’s disease and other inherited diseases, hyperlipoproteinemia, light chain deposition disease, chronic myelogenous leukemia, immune thrombocytopenic purpura, follicular (mineral oil) lipidosis (due to lipid in packaging of food, Hum Path 1984;15:724)

Ceroid:

benign histiocytes with abundant, foamy, vacuolated cytoplasm due to ingestion of sphingomyelin and other phospholipids
Sea-blue histiocyte syndrome (OMIM 269600): autosomal recessive, may be related to adult Niemann-Pick disease (Hum Path 1982;13:1115)

Micro: histiocytes diffusely within splenic cords; may separate the white pulp but don’t diminish its size

Positive stains:
fat stains, acid-fast stains, PAS diastase


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Gaucher cells:
large:

30–100μm diameter

nuclei:

one or more
central or eccentric

cytoplasm:

characteristic fibrillary or striated pattern
pale blue–gray color

H&E sections:

slightly eosinophilic
fibrillary pattern may be very prominent

The cytoplasm of many Gaucher cells has a fibrillary or granular appearance. Nuclei are small and usually eccentric in location.

variably and often intensely positive with PAS
stain positively for iron in older children and adults


Sea-blue Histiocyte Syndrome

Macrophages contain substance that stains:
blue in Romanowsky-stained smears
yellow to tan in H & E sections
has positive reactions with:
PAS
Sudan black B

sometimes appears to be ceroid


may be observed in several unrelated disorders and lacks diagnostic specificity including:

some cases of Niemann–Pick disease

Macrophages may resemble macrophages in Whipple's disease:

rarely detected in marrow
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