Elleder M
Hlava's First Institute of Pathology, First Medical Faculty, Charles University, Prague, CSFR.
Zentralbl Pathol. 1992;138(1):47-50.
Two cases of Gaucher's disease (Types I and III) are described with a substantial part of the storage cell population in several organs (liver, adrenal cortex) intensively vacuolized and transformed into non-specific foam cells. The transformation process encompassed massive three-dimensional distension of the lysosomal system filled with typical lipid tubules as well as severe reduction of the intervening cytoplasm and decrease or disappearance of histochemically detectable enzyme activities, high in typical Gaucher cell (GC). Such an extensive GC foamy transformation may cause diagnostic embarrassment in microscopic interpretation especially of liver biopsies.
本文描述了两例戈谢病(I型和III型),多个器官(肝脏、肾上腺皮质)中的大部分储存细胞群出现大量空泡化,并转化为非特异性泡沫细胞。转化过程包括充满典型脂质小管的溶酶体系统的大量三维扩张,以及中间细胞质的严重减少和组织化学可检测到的酶活性的降低或消失,而在典型的戈谢细胞(GC)中该酶活性较高。这种广泛的GC泡沫样转化可能会在显微镜检查尤其是肝脏活检的解读中造成诊断困难。