Light-chain deposition disease: Its relation with AL-type amyloidosis

Abstract

This review is based on our own observations of 14 patients with LCDD at Necker Hospital between 1973 and 1982 and on the data in the literature. Several of our case reports have already been published, together with a series from Tenon Hospital in Paris. In plasma cell dyscrasias (including myeloma, LCDD, and AL amyloidosis), the clinical features depend critically on the substances released by the 'dysregulated' plasma cell clone: for example, osteoclast activating factor in some myeloma patients with severe bone involvement; potentially nephrotoxic monoclonal light chains in Bence Jones myeloma leading to the classical myeloma kidney; abnormal light chains prone to tissue deposition in LCDD or light chains highly susceptible to proteolysis in macrophage lysosomes in AL amyloidosis. Further investigations are needed to clarify the cell defects leading to such a synthesis, as well as the intermediary mechanisms involved.