AL型心肌淀粉样变致心力衰竭的机制探讨

The mechanisms of heart failure caused by primary amyloid light chain cardiac amyloidosis

免疫球蛋白轻链型(AL型)心肌淀粉样变,是由骨髓中浆细胞分泌克隆性免疫球蛋白轻链在心肌间质等部位聚集,引发的以心力衰竭(心衰)、心律失常和心肌缺血为主要表现的一种疾病。心衰是AL型心肌淀粉样变终末期表现,但目前AL型心肌淀粉样变导致心衰的机制尚未被完全阐明。本文重点探讨了目前已知的AL型心肌淀粉样变导致心衰的机制,主要包括淀粉样原纤维的占位效应和毒性作用、游离轻链的毒性作用、冠脉微血管损伤、细胞外基质稳态破坏、传导系统损伤,旨在为未来进一步的机制研究和临床治疗探索提供参考。

Amyloid light chain(AL)amyloidosis is a clinical syndrome caused by the aggregation of clonal immunoglobulin light chains secreted by bone marrow plasma cells in myocardial interstitial sites which induces heart failure,arrhythmia and myocardial ischemia.Heart failure is the end-stage of AL cardiac amyloidosis,but the mechanism of heart failure due to AL cardiac amyloidosis has not been fully elucidated.This article summarizes the mechanism of it which includes amyloid fibrils occupying effect and toxic effect,free light chain toxic effect,coronary microvascular injury,extracellular matrix homeostasis disruption,conduction system damage,aiming to provide reference for further research and clinical treatment exploration.