Beyond the plasma cell: emerging therapies for immunoglobulin light chain amyloidosis

作者:Weiss Brendan M*; Wong Sandy W; Comenzo Raymond L
来源:Blood, 2016, 127(19): 2275-2280.
DOI:10.1182/blood-2015-11-681650

摘要

Systemic immunoglobulin light chain (LC) amyloidosis (AL) is a potentially fatal disease caused by immunoglobulin LC produced by clonal plasma cells. These LC form both toxic oligomers and amyloid deposits disrupting vital organ function. Despite reduction of LC by chemotherapy, the restoration of organ function is highly variable and often incomplete. Organ damage remains the major source of mortality and morbidity in AL. This review focuses on the challenges posed by emerging therapies that may limit the toxicity of LC and improve organ function by accelerating the resorption of amyloid deposits.

  • 出版日期2016-5-12