An Autopsied Case of Systemic Light Chain Deposition Disease (LCDD): The Relationship between LCDD and AL Amyloidosis
A case of the systemic light chain deposition disease in a 48-year-man is presented. The patient showed antemortem the signs of multiple organ failure, but without that of myeloma. Autopsy revealed generalized of hyaline-eosinophilic, Congo red negative substance. It was ultra-structurally differed from amyloid fibril. In bone marrow, myelomatous proliferations of plasma cells positive for kappa light chain were abundant. Deposited substance was identified as kappa light chain by immuno-histochemical and immunoelectron method. Biochemical and immunohistochemical findings on LCDD proteins extracted from the liver tissues, suggested that degradation process and amino acid composition and antigenecity of LCDD protein resembled to those of the AL proteins in AL amyloidosis.
KeywordsTyrosine Anemia Lysine Arginine Methionine
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