Ng I O, Sturgess R P, Williams R, Portmann B
Liver Unit, King's College Hospital, London, UK.
Histopathology. 1990 Aug;17(2):109-15. doi: 10.1111/j.1365-2559.1990.tb00680.x.
We report two adult patients whose liver biopsy specimens revealed numerous ground-glass hepatocytes due to inclusions resembling Lafora bodies. The inclusions were large, intracytoplasmic, pale, eosinophilic and kidney-shaped and were periodic acid-Schiff positive and HBsAg negative. Immunoperoxidase studies showed that the inclusions were positive for cytokeratins and alpha 1-antitrypsin. In case 1, the inclusions were not membrane-bound and consisted of secondary lysosomes and degenerate organelles including rough and smooth endoplasmic reticulum. In case 2, electronmicroscopy showed the inclusions were not membrane-bound, but consisted of dense granules, fibrils and vacuoles, with appearances very similar to Lafora bodies. Neither patient had myoclonus or epilepsy. Electronmicroscopy is important in differentiating the type of Lafora body like inclusions found in liver biopsies.
我们报告了两名成年患者，其肝脏活检标本显示由于存在类似拉福拉小体的包涵体而有大量毛玻璃样肝细胞。这些包涵体较大，位于细胞质内，呈淡色、嗜酸性且呈肾形，对过碘酸希夫反应呈阳性而对乙肝表面抗原呈阴性。免疫过氧化物酶研究表明，这些包涵体对细胞角蛋白和α1-抗胰蛋白酶呈阳性。在病例1中，包涵体无膜包裹，由次级溶酶体和包括粗面和滑面内质网在内的退化细胞器组成。在病例2中，电子显微镜显示包涵体无膜包裹，但由致密颗粒、纤维和空泡组成，其外观与拉福拉小体非常相似。两名患者均无肌阵挛或癫痫。电子显微镜对于区分肝脏活检中发现的类似拉福拉小体的包涵体类型很重要。
