Physiology, Diagnosis and Treatment of Cardiac Light Chain Amyloidosis

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.

Synthesis and evaluation of Fe-doped zinc oxide photocatalyst for methylene blue and congo red removal

“…In the textile industry, these compounds are mainly used to dye various textiles such as cotton, silk and wool. Other than the textile industry, methylene blue is also used in the pharmaceutical industry where it is commonly used as an antimalarial agent and antifungal agent for the treatment of diseases such as malaria and Alzheimer's, while congo red is also heavily used in histological studies and as an acid-base indicator due to its ability to turn red and blue in alkaline and acidic solution respectively [4,5]. Although methylene blue and congo red are significant to various industries especially the textile industry, these compounds are toxic and non-biodegradable which leads to various adverse health and environmental effects if left untreated in the wastewater [6].…”

Cardiac Amyloidosis in Autopsy Case of Sudden Unexpected Death

“…cardiac amyloidosis shows conduction abnormalities in approximately 50% of the patients [9]. Cardiac amyloidosis may cause insidious congestive heart failure in the case of interstitial amyloid deposits and produce a restrictive pattern of cardiomyopathy that may masquerade as chronic constrictive pericarditis [4].…”

“…AL amyloidosis has the worst prognosis among all the types of systemic amyloidosis. The median survival time is eight months without proper treatment [9]. The clinical symptoms of AL amyloidosis depend on the damage to the affected tissue and organs but are usually non-specific: advanced organ changes most often affect the heart (71%), kidneys (58%), gastrointestinal tract (22%), nervous system (23%), liver (16%), and soft tissues (10%) [19].…”

“…Therefore, the deposits were called amyloids, a name that is firmly entrenched despite the fact that the deposits are unrelated to starch [8]. To date, 37 proteins and peptides that form amyloid deposits in humans have been identified [9]. Amyloid is deposited in the extracellular spaces of various tissues.…”

Diagnostic and Prognostic Values of Cardiopulmonary Exercise Testing in Cardiac Amyloidosis

“…They include heart failure supportive therapy, therapies inhibiting the production of amyloid precursor proteins, and novel strategies inhibiting amyloid fibril formation, amyloid deposits, or stabilizing precursor proteins. In AL amyloidosis, the aim of treatment is to eradicate the underlying plasma cell clone that produces excess light chains ( 41 ). Chemotherapy regimens and autologous stem cell transplantation have been adapted from treatment of multiple myeloma, a related plasma cell dyscrasia.…”