Treatment of Amyloid Light-Chain Amyloidosis

Amyloid light-chain amyloidosis (AL) is characterized by deposits of immunoglobulin κ or λ free light chains (FLCs) produced by clonal bone marrow plasma cells. FLCs transform into amyloid fibrils and accumulate progressively in multiple organs and tissues. The main targets are the heart and kidneys, while the liver, gastrointestinal tract, and nervous system are less commonly involved. New therapeutic strategies targeting the plasma cell clone have improved the outcome of patients with this condition. Due to the systemic nature of the disease and due to its presentation, the management of patients with AL amyloidosis must be multidisciplinary. Disease staging is a crucial step as it allows identifying patients who are candidates for autologous stem cell transplantation and other therapeutic options. Overall, drug treatment consists of therapeutic schemes similar to those used for multiple myeloma. In the case of recurrence, second- and third-line therapies are used, which are also based on drugs used for multiple myeloma.