Gaucher cells

but may be irregularly shaped, and their location within the cell is ex-centric (Mandlebaum and Downey 1916). The chromatin appears more dense at the rim of the nucleus (Fisher and Reidbord 1962), the nucleoli are inconspicuous. The question of cell division, previously the subject of controversy (Epstein 1924b, Rusca 1921), has now been settled in favor of a preponderance of amitosis (Pittaluga and Goyanes 1933, Cazal 1944). 

Reidbord 1962),which, according to Czitober et al. (1964), can be demonstrated in 80% of the GC. During the deposition of cerebrosides the viscosity of the cytoplasm increases (Erf 1938). 

The formation of GC from reticulum cells appears to be established (Schlagen-HAUFER 1907, Risel 1909, Mandlebaum and Downey 1916, Kraus 1920, Bloom 

Oberling 1926, Pick 1933). In addition, osteoblasts and fibroblast-like spindle cells of the bone marrow (Block and Jacobson 1948) and adventitial cells and histiocytes store cerebrosides and become GC (Pick 1926). 

Gott and Pexa (1964), with the use of a method described by Klima et al. (1956), could demonstrate numerous GC in the circulating blood of an affected adult. 

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The "crumpled tissue paper" appearance of the cytoplasm of Gaucher cells is caused by enlarged, elongated lysosomes filled with glucocerebroside. 

Figure 9 Effect of jnaimose-tenmnal ghicocerefanMidasc on the bone morrow. Right posterior iliac crest biopsy specimens were obtained before (Pre) and after (Post) 3 1/2 yearn of treatment with bigh-dost enzyme supplementation. Marked clearing of the Gaucher cell in Si bate is evident in the pOHttxcalment specimen. Original magnification XJJ. (Frora Ref. 28.)...

Figure 16-1. Wright-stained Gaucher cells obtained from bone marrow showing the typical wrinkled appearance of the cytoplasm (magnification 175x).

Figure 16-3. Splenic Gaucher cells, light micrograph.

The storage cells in the bone marrow have a myelophthisic effect that is, they crowd out the normal hematopoietic tissues. As a result of Gaucher cell accumulation in the bone marrow,... 

Figure 16-7. An electron micrograph of the typical lysosomal inclusions of a splenic Gaucher cell.

Fig. 31.15 Gaucher s disease. Gaucher cells (sphingolipid-storing macrophages) within the liver parenchyma (arrow), shown here as pale-blue cells with an internal structure similar to cigarette paper (PAS)...

Type III (chronic juvenile neurological type) At the onset of the disease, splenomegaly and, subsequently, hepatomegaly are observed. Gaucher cells are found in the bone marrow at an early stage. There are severe cerebral and neurological disorders. 

F. P. Castronovo, K. A. McKusick, S. H. Doppelt, and N. W. Barton. Radiopharmacology of inhaled l33Xe in skeletal sites containing deposits of Gaucher cells. Nucl. Med. Biol. 20 707 (1993). 

Lee et al. (1977) in a child aged 2 /2 years observed a remarkable number of Gaucher cells in alveolar space and in terminal bronchioles heavily laden with iron. 

Ultrastructural studies on spleen biopsies of the Gaucher cell have shown the presence of ovoid, irregular cytoplasmic bodies, which correspond to the stria-tion described by the light microscopist (see Fig. 3-35). Degenerate mitochondria were found in close association with these bodies, and it was postulated that the accumulating material was in some way derived from mitochondria. Vesicles of the endoplasmic reticulum were also present in these areas, and the possibility that the lipoid material was incorporated in areas of focal cytoplasmic degradation was not excluded [120]. 

Twenty-five per cent of patients with Gaucher s disease have a typical eye injury—a wedge-shaped, yellowish streak with its base directed inward close to the cornea and its apex directed outward toward the canthus. Although one would suspect that this anomaly results from the conglomeration of Gaucher cells in the sclera, no such cells have been detected on histological examination. 

Kattlove and his associates believe that the glucocerebroside which accumulates in Gaucher s disease is principally derived from polymorphonuclear cells which have a rapid turnover and are rich in ceramide lactoside [122]. (This explains the occasional presence of Gaucher cells in marrow of patients with chronic myelogenous leukemia.)... 

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