Liver involvement in nonamyloid light chain deposits disease.
Liver examination performed in seven patients who had renal failure related to light chain deposits demonstrated in all cases the presence of liver light chain deposits. In all of our patients clinical renal involvement antedated the liver disease. The portal areas and the Disse spaces contained a granular material which strongly reacted with antilight chain antiserum (kappa or gamma). In one patient in whom lesions were severe, the sinusoid edge was ruptured and a pelliosis -like lesion was observed. In the five patients who were hemodialyzed for more than several months at the time of discovery of liver deposits, increased amounts of collagen were present in the Disse spaces, and one patient had extensive liver fibrosis by light microscopy. Clinical liver involvement was defined by moderate hepatomegaly in five patients, with ascites in two. A slight increase in phosphatase alkaline activity was frequently observed and bromsulphalein retention was present in two. In one patient liver tests remained entirely normal despite the presence of diffuse kappa light chain deposits.
D000368:Aged; D005455:Fluorescent Antibody Technique; D006801:Humans; D006942:Hypergammaglobulinemia / Q000276:immunology*; D007147:Immunoglobulin Light Chains / Q000032:analysis*; D007145:Immunoglobulin kappa-Chains / Q000032:analysis*; D007146:Immunoglobulin lambda-Chains / Q000032:analysis; D007676:Kidney Failure, Chronic / Q000276:immunology; D008099:Liver / Q000648:ultrastructure*; D008107:Liver Diseases / Q000276:immunology*; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains