Amyloid Protein in AL-Amyloidosis. Natural History and Potential for Regression
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Abstract
Amyloid light chain amyloidosis is the most commonly seen form of systemic amyloidosis and is characterized by the accumulation of circulating monoclonal immunoglobulin light chain precursors arising from an abnormal clone of plasma cells. These light chain precursors demonstrate a propensity for misfolding, aggregation and deposition as highly stable fibrillar cross-linked beta sheets in various tissues and organs in a manner characteristic of other forms of systemic amyloidosis. There have been great advances in the treatment of the underlying plasma cell dyscrasia with improved long-term survival but the persistence of extracellular amyloid deposits in the tissues of vital organs, particularly the heart and kidney remains a significant cause of morbidity and mortality even in those patients in whom a hematological complete response has been achieved. This review focuses on the importance of early diagnosis and treatment on limiting the extent of amyloid protein accumulation and organ dysfunction and the current state of knowledge on the potential for resorption and clearance of these amyloid deposits in patients with amyloid light chain amyloidosis. The status of current novel anti-fibrillar agents is reviewed and future strategies for effectively intervening to improve organ function and survival these individuals is discussed.
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