肝脏内质网储存疾病：胞质内透明包涵体的特征

Endoplasmic storage disease of liver: characterization of intracytoplasmic hyaline inclusions.

作者信息

Ng I O, Ng M, Lai E C, Wu P C

机构信息

Department of Pathology, University of Hong Kong, Queen Mary Hospital.

出版信息

Histopathology. 1989 Nov;15(5):473-81. doi: 10.1111/j.1365-2559.1989.tb01607.x.

DOI:10.1111/j.1365-2559.1989.tb01607.x

PMID:2480934

Abstract

Two cases of endoplasmic storage disease of liver are described. The liver tissue in each case showed numerous intracytoplasmic hyaline inclusions of varying sizes with formation of ground-glass hepatocytes. These inclusions were pale eosinophilic in hematoxylin & eosin stained sections, and were periodic acid-Schiff and HBsAg negative. Immunoperoxidase studies revealed strong positivity for fibrinogen and complement components C3 and C4 in case 1 and exclusive positivity for fibrinogen in case 2. On electron microscopy, the inclusions appeared as granular or fibrillar material within dilated cisternae of rough endoplasmic reticulum.

摘要

本文描述了两例肝脏内质网储存疾病。每例肝脏组织均显示出大量大小不一的胞浆内透明包涵体，形成毛玻璃样肝细胞。这些包涵体在苏木精-伊红染色切片中呈淡嗜酸性，过碘酸-希夫反应及乙肝表面抗原均为阴性。免疫过氧化物酶研究显示，病例1中纤维蛋白原、补体成分C3和C4呈强阳性，病例2中仅纤维蛋白原呈阳性。电镜下，包涵体表现为粗面内质网扩张池内的颗粒状或纤维状物质。