Abstract
There are reports indicating that myocardial dysfunction in systemic immunoglobulin light chain amyloidosis (AL amyloidosis) stems not only from the amyloid deposit in the organ but also the cardiotoxicity of the amyloid precursor free light chains (FLCs) circulating in the blood. The aim of the study is to analyze the role of sFLC κ and λ in the assessment of heart involvement and the degree of myocardial damage in AL amyloidosis. The study involved 71 patients diagnosed with primary AL amyloidosis. The relationship between sFLC concentrations and cardiac biochemical and echocardiographic parameters was assessed. The median concentrations of N-terminal pro b-type natriuretic peptide(NT-proBNP) and troponin I (TnI) were significantly higher in patients with amyloids formed from monoclonal λ chains compared to patients with monoclonal κ proliferation. In patients with heart involvement by amyloids formed from monoclonal FLC, the study demonstrated a statistically significant positive correlation between the concentration of monoclonal antibody λ chain and TnI (R = 0.688; p < 0.05), NT-proBNP (R = 0.449; p < 0.05), and the value of diastolic dimension of the interventricular septum (IVS; R = 0.496, p < 0.05). The above data indicate that the presence of monoclonal λ chains in patients with AL amyloidosis may be associated with more severe damage to cardiomyocytes and dysfunction of the myocardium.
Highlights
Light chain amyloidosis (immunoglobulin light chain amyloidosis (AL)) is caused by clonal neoplastic plasma cells or B-cell clones that produce misfolded light chains (κ or λ)
Different clinical features, and diagnostic limitations are common causes of late diagnosis [4]. It should be included in the differential diagnosis of the following five syndromes: nephrotic syndrome in patients who have not been treated for diabetes; cardiomyopathy confirmed by echocardiography, which is not the result of myocardial ischemia; hepatomegaly or increased alkaline phosphatase activity; neuropathy with the presence of monoclonal protein (M) in serum; monoclonal gammopathy of undetermined significance (MGUS), accompanied by weight loss, unexplained fatigue, peripheral edema, or paresthesia [3]
AL diagnosis criteria include (1) presence of organ abnormalities resulting from amyloid deposition; (2) presence of amyloid confirmed by Kongo red staining in tissue biopsy or organ biopsy; (3) evidence that amyloid is composed of light chain immunoglobulins; (4) diagnosis of plasma cell dyscrasia (confirming the presence of monoclonal protein in serum and/or in urine or clonal plasma cells in bone marrow, and an abnormal free light chain (FLC) κ/λ ratio- 1.65)
Summary
Light chain amyloidosis (immunoglobulin light chain amyloidosis (AL)) is caused by clonal neoplastic plasma cells or B-cell clones that produce misfolded light chains (κ or λ). Different clinical features, and diagnostic limitations are common causes of late diagnosis [4] It should be included in the differential diagnosis of the following five syndromes: nephrotic syndrome in patients who have not been treated for diabetes; cardiomyopathy confirmed by echocardiography, which is not the result of myocardial ischemia; hepatomegaly (with a proper image of its parenchyma assessed in imaging studies) or increased alkaline phosphatase activity; neuropathy with the presence of monoclonal protein (M) in serum; monoclonal gammopathy of undetermined significance (MGUS), accompanied by weight loss, unexplained fatigue, peripheral edema, or paresthesia [3]. The latest risk stratification, formulated by Kumar et al in 2012, is based on NT-proBNT, cardiac troponin T (TnT), or troponin I (TnI) concentrations, and the difference between the concentration of involved and uninvolved free light chains (dFLCs), the value of which differs between cancer process (monoclonal, amyloidogenic) and nonmalignant hypergammaglobulinemia [5]
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