Amyloidosis of immunoglobulin light chains (AL-amyloidosis)

Amyloidosis is a general term for a group of diseases that are caused by the misfolding of proteins and their deposition in organs and tissues in the form of an insoluble protein fibrils. The base of amyloid is the fibril that built up by twisted protein protofilaments. AL amyloidosis is one the form of plasma cell disorders, characterized by overproduction of misfolded immunoglobulin light chains by clonal plasma cells or В- lymphocytes. Survival in AL amyloidosis depends on the spectrum of organ involvement, the severity of organs involved and hematological response to treatment. Light chain fibrils infiltrate the myocardium, interfere with cell–cell coupling, disrupt cellular integrity and may contribute to cell injury and sudden death. That is why the cardiac involvement is the main predictor of patient outcome. The modern approach to diagnosis and treatment based on identification the most vulnerable group of patients with stage IIIb heart disease. The main goal of therapy is rapidly and profoundly suppress the production of amyloidogenic light chains. For a long time, therapy with melphalan and autologous hematopoietic stem cell transplantation considered a standard of care in AL amyloidosis, but the addition of the targeted drugs - bortezomib, daratumumab and venetoclax increased the efficacy of treatment dramatically.