Bedossa P, Fabre M, Paraf F, Martin E, Lemaigre G
Service d'Anatomie Pathologique, Hôpital Antoine Béclère, Clamart, France.
Hum Pathol. 1988 Sep;19(9):1008-14. doi: 10.1016/s0046-8177(88)80078-9.
Light and transmission electron microscopic study as well as immunohistochemical investigation were performed on three cases of light chain deposition disease (LCDD) with severe liver dysfunction. In two cases, the amount of light chain deposits in the liver was moderate and did not correlate with the severity of clinical and biological symptoms. Ultrastructural study demonstrated a collagenization of the Disse's space, with basement membrane-like material in association with light chain deposits. Immunohistochemical investigation showed a marked increase of collagen types I, III, and IV, as well as fibronectin and laminin in perisinusoidal space. This study suggests that collagenization of the Disse's space has a minor role in liver dysfunction. The analogy between kidney and liver lesions in diabetes and in LCDD is stressed, but the mechanism of this abnormal accumulation of matrix proteins remains unknown.
对三例伴有严重肝功能障碍的轻链沉积病（LCDD）进行了光镜和透射电镜研究以及免疫组化研究。在两例病例中，肝脏中轻链沉积物的量为中度，且与临床和生物学症状的严重程度无关。超微结构研究显示狄氏间隙出现胶原化，伴有与轻链沉积物相关的基底膜样物质。免疫组化研究表明，肝血窦周间隙中I型、III型和IV型胶原以及纤连蛋白和层粘连蛋白显著增加。本研究提示狄氏间隙的胶原化在肝功能障碍中作用较小。强调了糖尿病和LCDD中肾脏与肝脏病变之间的相似性，但基质蛋白这种异常蓄积的机制仍不清楚。
