Ceroid-lipidosis: an acquired storage-type disease of liver and hepatic lymph node.

Abstract

The term is used to describe the phenomenon of accumulation of abnormal amounts of neutral fat in cells consequent to certain nutritional, toxic or anoxic derangements. In contrast, its plural generally is used to denote a group of inherited storage diseases in which incompletely degraded sphingolipids accumulate within lysosomes as a result of a deficiency of a specific lysosomal hydrolase [3]. In this context its use may be extended to include the inherited ceroid-lipofuscinoses 13, 7] and the iatrogenic lipidoses caused by administration of various cationic amphiphilic drugs such as chloroquine ll]. This report describes a form of lipidosis which was seen in the livers and associated lymph nodes of four dogs of different breeds and a cat; it had features in common with the lipidoses mentioned above. The abnormalities were all incidental necropsy findings in animals with other diseases. At necropsy, all livers had various degrees of pallor and contained numerous disseminated, yellow-white foci 1.0 mm in diameter. Cut surfaces of the livers bulged slightly and looked greasy. In each instance, one lymph node identified as hepatic node or believed to be hepatic node on retrospective study of the postmortem report, was conspicious for its moderate enlargement and 1.0 to lO-mm rounded protuberances. When cut, the nodules were yellow, gritty and slightly greasy. Histologically, there was mild to moderate fatty change in three of the livers and severe fatty change in the liver from one dog. The characteristic feature common to all livers was numerous, discrete, focal deposits of lipid material that either had not, or only partially had been extracted in routine preparation of paraffin sections (fig. I). This lipid material was contained in macrophages which often were present as polykaryons. The lymph nodes were infiltrated heavily with foamy macrophages containing lipid material which had the same appearance and staining characteristics as that seen in macrophages in the liver. A large proportion of the macro phages in lymph nodes also were polykaryons (fig. 2). In hematoxylin and eosin-stained sections, the abnormal lipopigment was slightly opaque and varied in color from almost colorless-gray to gray-yellow, yellow, and yellow-brown. It stained positively with Sudan black, periodic acid-Schiff, Ziehl-Neelsen, and luxol fast blue stains. Perl's stain revealed the presence of various amounts of iron in the stored material in four of the five livers. Unstained, deparaffinized sections examined with blue light showed the storage lipopigment to be strongly autofluorescent in all livers. Ultrastructurally, macrophages and their polykaryons contained numerous heterogeneous globoid bodies of various sizes, and some clearly were contained within a membrane. Electron density varied between and within bodies from a partially opaque lipid-like material, to areas of dense granular, sometimes slightly membranous, material (fig. 3). The histological appearance of stored material within macrophages and macrophage polykaryons was similar to lesions in certain of the inherited sphingolipidoses such as