Abstract: Systemic light chain (AL) amyloidosis is a rare plasma cell disease that often causes dysfunction of important organs such as heart and kidney. In recent years, some progress has been made in the field of molecular mechanism of AL amyloidosis, which helps us further understand structural characteristics of amyloid fibrils in AL amyloidosis and pathological mechanism of heart and kidney damage; In addition, there has been some advances in diagnosis and treatment of AL amyloidosis, which is of great significance for improving clinical practice of AL amyloidosis. This article will review recent progress in pathogenesis, diagnosis and treatment of AL amyloidosis.

Key words: systemic light chain amyloidosis, pathogenesis, diagnosis, molecular markers, immunotherapy