Light chain deposition disease with liver dysfunction.
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.
D000368:Aged; D003094:Collagen / Q000032:analysis; D005260:Female; D005353:Fibronectins / Q000032:analysis; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007150:Immunohistochemistry; D008107:Liver Diseases / Q000150:complications* / Q000378:metabolism; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D010265:Paraproteinemias / Q000378:metabolism* / Q000503:physiopathology
D005353:Fibronectins; D007147:Immunoglobulin Light Chains; D003094:Collagen