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AL Amyloidosis: Current Chemotherapy and Immune Therapy Treatment Strategies : JACC: CardioOncology State-of-the-Art Review

ABSTRACT: Immunoglobulin light chain (AL) amyloidosis is an incurable plasma cell disorder characterized by deposition of fibrils of misfolded immunoglobulin free light chains (FLC) in target organs, leading to failure. Cardiac involvement is common in AL amyloidosis and represents the single most adverse prognostic feature. A high index of clinical suspicion with rapid tissue diagnosis and commencement of combinatorial, highly effective cytoreductive therapy is crucial to arrest the process of amyloid deposition and preserve organ function. The clinical use of molecularly targeted drugs, such as proteasome inhibitors and immunomodulatory agents, monoclonal antibodies such as daratumumab, and risk-adjusted autologous stem cell transplant in eligible patients, has radically changed the natural history of AL amyloidosis. Here, we review the state-of-the-art treatment landscape in AL amyloidosis with an eye toward future therapeutic venues to impact the outcome of this devastating illness. Central Illustration Highlights • Cardiac involvement in AL amyloidosis is common and represents the single most adverse prognostic factor.• Chemo-immunotherapies and autologous stem cell transplant lead to prolonged remission and survival in low-stage patients.• Early diagnosis is critical in AL amyloidosis to avoid irreversible organ damage.• Rapid and deep FLC reduction is necessary to ensure long-term survival and functional recovery of affected organs.

SUBMITTER: Bianchi G

PROVIDER: S-EPMC8543128 | biostudies-literature |

REPOSITORIES: biostudies-literature

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