Pathologic immunoglobulin deposition can lead to significant organ dys
function, morbidity and mortality. This article examines the recent ad
vances in the understanding of both host and protein factors in the pa
thogenesis of primary systemic amyloidosis and light chain deposition
disease. Limitations of current therapy are reviewed and newer approac
hes including iododoxorubicin, dexamethasone, and high-dose therapy ar
e discussed.