Czyżewska Emilia, Wiśniewska Agnieszka, Waszczuk-Gajda Anna, Ciepiela Olga
Department of Laboratory Medicine, Medical University of Warsaw, Banacha 1a, 02-097 Warsaw, Poland.
Department of Hematology, Oncology and Internal Diseases, Medical University of Warsaw, Banacha 1a, 02-097 Warsaw, Poland.
J Clin Med. 2021 Mar 18;10(6):1274. doi: 10.3390/jcm10061274.
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; < 0.05), NT-proBNP (R = 0.449; < 0.05), and the value of diastolic dimension of the interventricular septum (IVS; R = 0.496, < 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.
有报告表明，系统性免疫球蛋白轻链淀粉样变性（AL淀粉样变性）中的心肌功能障碍不仅源于器官中的淀粉样蛋白沉积，还源于血液中循环的淀粉样蛋白前体游离轻链（FLC）的心脏毒性。本研究的目的是分析可溶性FLC κ和λ在评估AL淀粉样变性中心脏受累情况及心肌损伤程度方面的作用。该研究纳入了71例诊断为原发性AL淀粉样变性的患者。评估了可溶性FLC浓度与心脏生化及超声心动图参数之间的关系。与单克隆κ增殖患者相比，由单克隆λ链形成淀粉样蛋白的患者中，N末端B型利钠肽原（NT-proBNP）和肌钙蛋白I（TnI）的中位浓度显著更高。在由单克隆FLC形成淀粉样蛋白而累及心脏的患者中，研究显示单克隆抗体λ链浓度与TnI（R = 0.688；P<0.05）、NT-proBNP（R = 0.449；P<0.05）以及室间隔舒张期内径值（IVS；R = 0.496，P<0.05）之间存在统计学显著正相关。上述数据表明，AL淀粉样变性患者中存在单克隆λ链可能与更严重的心肌细胞损伤和心肌功能障碍有关。
