κ 和 λ 轻链淀粉样变的病理评分系统分析差异。

Differences between κ and λ light chain amyloidosis analyzed by a pathologic scoring system.

机构信息

Department of Pathology, School of Basic Medical Sciences, Fudan University, Shanghai, China.

Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.

出版信息

Am J Clin Pathol. 2023 Aug 1;160(2):144-149. doi: 10.1093/ajcp/aqad017.

DOI:10.1093/ajcp/aqad017

PMID:36933186

Abstract

OBJECTIVES

Amyloid light chain (AL)-κ and AL-λ share common histopathologic changes; however, the potential difference in clinical manifestations, histologic findings, and clinical significance between the 2 subtypes remain unclear.

METHODS

In a retrospective study, 94 kidney biopsies for AL amyloidosis were evaluated using the composite scarring injury score (CSIS) and amyloid score (AS). Results were then compared between AL-κ and AL-λ.

RESULTS

Comparing AS and CSIS between AL-κ and AL-λ, the AS was significantly higher in AL-κ than in AL-λ, with 2 components of AS (capillary wall and vascular amyloid) scoring higher in AL-κ than in AL-λ, while mesangial and interstitial ASs were similar in the 2 cohorts. In addition, the proportion of periodic acid-Schiff strong-staining amyloid in AL-κ was markedly higher than in AL-λ. There was no significant difference in CSIS and its components between the 2 subtypes of AL amyloidosis.

CONCLUSIONS

Overall, AL-κ presents with higher serum creatinine and a higher AS score than AL-λ at biopsy, which may indicate a worse prognosis and be an important reference for clinical management.

摘要

目的

轻链κ（AL-κ）和轻链 λ（AL-λ）淀粉样变共享常见的组织病理学改变；然而，这两种亚型在临床表现、组织学发现和临床意义方面的潜在差异仍不清楚。

方法

在一项回顾性研究中，使用复合瘢痕损伤评分（CSIS）和淀粉样评分（AS）对 94 例 AL 淀粉样变性的肾活检进行评估。然后比较 AL-κ 和 AL-λ 之间的结果。

结果

比较 AL-κ 和 AL-λ 之间的 AS 和 CSIS，AL-κ 的 AS 明显高于 AL-λ，AS 的 2 个组成部分（毛细血管壁和血管淀粉样变）在 AL-κ 中的评分高于 AL-λ，而系膜和间质 AS 在两组中相似。此外，AL-κ 中过碘酸-希夫强染色淀粉样物质的比例明显高于 AL-λ。两种类型的 AL 淀粉样变在 CSIS 及其组成部分之间没有显著差异。