Heavy-Chain Deposition Disease

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Abstract
Light-chain amyloidosis and light-chain deposition disease are closely related processes characterized by tissue deposition of organized (fibrillar) or nonorganized material related to monoclonal immunoglobulin light chains. Both conditions can occur in patients with immunoproliferative disorders. The clinical manifestations of light-chain deposition disease result from multivisceral involvement and are heterogeneous. Rapidly progressive renal failure or the nephrotic syndrome is the most common manifestation, but heart failure, arrhythmia, liver involvement (peliosis), and neurologic disorders may also be present. These findings may be caused by structural abnormalities or unusual behavior of deposited light chains. In particular, pathogenic light chains are characterized by unusual sizes, glycosylation, variable-region subgroups, exposed hydrophobic amino acid residues, or the propensity to polymerize or aggregate1. Soon after the first description of light-chain deposition disease,2 it became apparent that heavy chains that are monoclonal in origin may be found together with light chains in the tissue of some patients3.
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