Abstract
AL (light-chain) amyloidosis is a systemic disease in which amyloid fibers are formed from kappa or lambda immunoglobulin light chains, or fragments thereof, produced by a neoplastic clone of plasmocytes. The produced protein is deposited in tissues and organs in the form of extracellular deposits, which leads to impairment of their functions and, consequently, to death. Despite the development of research on pathogenesis and therapy, the mortality rate of patients with late diagnosed amyloidosis is 30%. The diagnosis is delayed due to the complex clinical picture and the slow progression of the disease. This is the type of amyloidosis that most often contributes to cardiac lesions and causes cardiac amyloidosis (CA). Early diagnosis and correct identification of the type of amyloid plays a crucial role in the planning and effectiveness of therapy. In addition to standard histological studies based on Congo red staining, diagnostics are enriched by tests to determine the degree of cardiac involvement. In this paper, we discuss current diagnostic methods used in cardiac light chain amyloidosis and the latest therapies that contribute to an improved patient prognosis.
Highlights
Systemic amyloidosis is a disease caused by the deposition of abnormally folded fibrous proteins in extracellular spaces in various tissues and organs [1]
The test that is characterized by the highest sensitivity in the diagnosis of AL amyloidosis is the determination of free light chains (FLC) in serum (88%), and additional urine immunofixation increases the sensitivity to 98%
AL-cardiac amyloidosis (CA) is mostly detected at an advanced stage of the disease, which is associated with the presence of organ complications at the time of diagnosis
Summary
Systemic amyloidosis is a disease caused by the deposition of abnormally folded fibrous proteins in extracellular spaces in various tissues and organs [1]. Two types of amyloidosis are known to infiltrate this organ: light chain amyloidosis (AL), transthyretin amyloidosis (ATTR), and sometimes, in acquired amyloidosis, type A (AA) cardiac involvement may occur [3]. AL, formerly called primary amyloidosis, is a clonal disorder of plasma cells caused by overproduction and abnormal folding of antibody light chain fragments [4]. Cardiac involvement occurs through extracellular amyloid infiltration in the myocardium, which causes thickening of the walls of both chambers. This contributes to excessive fluid accumulation in the body known as congestive heart failure [5]. This review paper will discuss the pathophysiology of cardiac light chain amyloidosis and the current methods used to diagnose and treat this condition
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