单克隆蛋白的延迟鉴定与孤立性心脏 AL 淀粉样变性的早期死亡有关。

IF 5.2 2区 医学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY
Paolo Milani, Francesca Fabris, Roberta Mussinelli, Giuseppe Damiano Sanna, Marco Basset, Pietro Benvenuti, Claudia Bellofiore, Martina Nanci, Mario Nuvolone, Andrea Attanasio, Gianluigi Guida, Stefano Perlini, Andrea Foli, Giampaolo Merlini, Giovanni Palladini
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引用次数: 0

摘要

背景:由于免疫球蛋白轻链淀粉样变性(AL)进展迅速,因此及早发现至关重要。单克隆轻链(M-LC)检测是诊断疑似心脏淀粉样变性(CA)患者的第一步。我们的目的是确定首次怀疑 CA 与 M-LC 检测之间的时间间隔是否与 AL 淀粉样变性的生存结果有关:纳入2016年至2020年间在本中心确诊的所有孤立性心脏AL淀粉样变性患者(n = 94)。已存在已知单克隆蛋白(意义未定的单克隆丙种球蛋白病或多发性骨髓瘤)的患者除外。计算诊断检查和确诊的时间间隔,并评估其生存预测能力:结果:首次怀疑CA(超声心动图)与M-LC检测之间的时间间隔与早期死亡率相关,预测生存率的最佳临界值为6周。在首次怀疑后 6 周以上接受 M-LC 检测的 26 名患者(占整个队列的 28%)中,梅奥 IIIb 期患者的比例更高(65% 对 35%,P = .008),其总生存率低于早期接受 M-LC 检测的患者(68 人,72%)(中位 3 个月对 14 个月,P = .039):结论:单克隆蛋白检测应作为超声心动图/其他仪器红旗引起CA怀疑的患者诊断工作的第一步。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Delayed identification of monoclonal protein is associated with early death in isolated cardiac AL amyloidosis.

Background: Early identification of immunoglobulin light-chain amyloidosis (AL) is crucial due to its rapid progression. Monoclonal light-chain (M-LC) testing is the first step in the diagnostic workup for patients with suspected cardiac amyloidosis (CA). We aimed to determine whether the time interval between the first CA suspicion and M-LC testing can be related to AL amyloidosis survival outcomes.

Methods: All patients (n = 94) with isolated cardiac AL amyloidosis diagnosed at our center between 2016 and 2020 were included. Those with pre-existing known monoclonal protein (monoclonal gammopathy of undetermined significance or smoldering multiple myeloma) were excluded. Time intervals to diagnostic tests and diagnosis were calculated and assessed for their survival prediction ability.

Results: The time interval between first CA suspicion (on echocardiography) and M-LC testing correlated with early mortality, and the best cutoff predicting survival, was 6 weeks. The 26 patients (∼28% of entire cohort) who underwent M-LC-studies >6 weeks after first suspicion more frequently presented Mayo stage IIIb (65% vs. 35%, p = .008), showing poorer overall survival than those (n = 68, 72%) referred for early M-LC studies (median 3 vs. 14 months, p = .039).

Conclusions: Monoclonal protein testing should be the first-step in the diagnostic workup for patients with echocardiographic/other instrumental red flags raising CA suspicion.

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来源期刊
Amyloid-Journal of Protein Folding Disorders
Amyloid-Journal of Protein Folding Disorders 生物-生化与分子生物学
CiteScore
10.60
自引率
10.90%
发文量
48
审稿时长
6-12 weeks
期刊介绍: Amyloid: the Journal of Protein Folding Disorders is dedicated to the study of all aspects of the protein groups and associated disorders that are classified as the amyloidoses as well as other disorders associated with abnormal protein folding. The journals major focus points are: etiology, pathogenesis, histopathology, chemical structure, nature of fibrillogenesis; whilst also publishing papers on the basic and chemical genetic aspects of many of these disorders. Amyloid is recognised as one of the leading publications on amyloid protein classifications and the associated disorders, as well as clinical studies on all aspects of amyloid related neurodegenerative diseases and major clinical studies on inherited amyloidosis, especially those related to transthyretin. The Journal also publishes book reviews, meeting reports, editorials, thesis abstracts, review articles and symposia in the various areas listed above.
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