Recent Advances in the Diagnosis, Risk Stratification, and Management of Systemic Light-Chain Amyloidosis

摘要: The term amyloidosis refers to a group of disorders in which protein fibrils accumulate certain organs, disrupt their tissue architecture, and impair the function effected organ. clinical manifestations prognosis vary widely depending on specific type affected protein. Immunoglobulin light-chain (AL) is most common form systemic amyloidosis, characterized by deposition misfolded monoclonal that secreted from plasma cell clone. Demonstrating amyloid deposits biopsy stained with Congo red mandatory for diagnosis. Novel agents (proteasome inhibitors, immunomodulatory drugs, antibodies, venetoclax) autologous stem transplantation, used eliminating underlying clone, have improved outcome low- intermediate-risk patients, but high-risk patients still grave. Randomized studies evaluating antibodies target (PRONTO, VITAL) were recently stopped due futility currently there an intensive search novel treatment approaches AL amyloidosis. Early diagnosis paramount importance effective prognosis, progressive nature this disease.