Suppr超能文献

尽管完全血液学缓解,但缺乏肾脏反应的 AL 淀粉样变性患者重复肾活检的价值:病例报告及文献复习。

Value of repeat renal biopsy in the evaluation of AL amyloidosis patients lacking renal response despite of complete hematologic remission: a case report and literature review.

机构信息

Nephrology Department, Sichuan Provincial People's Hospital, Chengdu, 610072, China.

Medical School, University of Electronic Science and Technology of China, Chengdu, 611731, China.

出版信息

BMC Nephrol. 2022 Mar 31;23(1):127. doi: 10.1186/s12882-022-02752-4.

Abstract

BACKGROUNDS

Published literatures on repeat renal biopsy of AL amyloidosis have basically reached a consensus that amyloid material deposit does not disappear or diminish after satisfactory hematologic response, regardless of renal response. However, the need of a repeat renal biopsy in such situation is still controversial.

CASE PRESENTATION

Here we reported a case of histologically confirmed λ Type renal AL amyloidosis who had been classified as Stage I and low risk at initial diagnosis. The patient received a total of six courses of CyBorD chemotherapy. She had achieved complete hematologic remission after two courses of chemotherapy but consistently had large amount of proteinuria over 10 g/day during follow up. A repeat renal biopsy was performed nine months after the first one and indicated mild to moderate increase of amyloid deposits as well as significant glomerulosclerosis and interstitial lesions, suggesting a lack of histological renal improvement despite her satisfactory hematologic response.

CONCLUSIONS

This case indicated renal involvement in AL amyloidosis could progress after successful hematologic treatment, and supported the value of repeat renal biopsy in the evaluation of AL amyloidosis patients lacking renal response despite of complete hematologic remission.

摘要

背景

已发表的关于 AL 淀粉样变性重复肾活检的文献基本达成共识,即无论肾脏反应如何,在满意的血液学反应后,淀粉样物质沉积不会消失或减少。然而,在这种情况下是否需要重复肾活检仍存在争议。

病例介绍

本文报道了 1 例经组织学证实的 λ 型肾 AL 淀粉样变性患者,该患者在初始诊断时被归类为Ⅰ期和低危。该患者共接受了 6 个疗程的 CyBorD 化疗。她在两个疗程的化疗后达到完全血液学缓解,但在随访期间持续出现大量蛋白尿(>10 g/天)。第一次肾活检后 9 个月进行了重复肾活检,结果显示淀粉样物质沉积轻度至中度增加,以及明显的肾小球硬化和间质病变,提示尽管血液学反应良好,但组织学肾脏改善不足。

结论

本病例表明 AL 淀粉样变性患者在成功进行血液学治疗后肾脏受累仍可进展,支持在完全血液学缓解但缺乏肾脏反应的 AL 淀粉样变性患者中重复肾活检的价值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d1c/8974030/ca52fa0a0c31/12882_2022_2752_Fig1_HTML.jpg

相似文献

2
The utility of repeat kidney biopsy in systemic immunoglobulin light chain amyloidosis.
Amyloid. 2020 Mar;27(1):17-24. doi: 10.1080/13506129.2019.1672650. Epub 2019 Oct 9.
3
Immunoglobulin Light Chain Amyloidosis Presenting as Inferior Vena Cava Thrombosis.
Am Surg. 2023 Jun;89(6):2788-2790. doi: 10.1177/00031348211050834. Epub 2021 Nov 6.
4
Gastroduodenal Involvement in AL Amyloidosis: Case Report and Literature Review.
J Investig Med High Impact Case Rep. 2024 Jan-Dec;12:23247096241237759. doi: 10.1177/23247096241237759.
5
[Renal disorders associated with monoclonal gammopathies: diagnostic and therapeutic progress].
Presse Med. 2012 Mar;41(3 Pt 1):276-89. doi: 10.1016/j.lpm.2011.11.008. Epub 2012 Jan 13.
6
Outcome of patients with severe AL amyloidosis and biopsy-proven renal involvement ineligible for bone marrow transplantation.
J Nephrol. 2021 Feb;34(1):231-240. doi: 10.1007/s40620-020-00748-7. Epub 2020 May 29.

引用本文的文献

1
Enhancing prognostic guidance in renal light-chain amyloidosis: a new staging system incorporating pathological characters.
Int Urol Nephrol. 2025 Jan;57(1):275-283. doi: 10.1007/s11255-024-04182-7. Epub 2024 Aug 13.

本文引用的文献

1
[Investigation of the safety and usefulness of a renal biopsy for older elderly patients ≥75 years old].
Nihon Ronen Igakkai Zasshi. 2021;58(3):453-458. doi: 10.3143/geriatrics.58.453.
2
Safety of Renal Biopsy by Physicians with Short Nephrology Experience.
Healthcare (Basel). 2021 Apr 16;9(4):474. doi: 10.3390/healthcare9040474.
3
Management of AL amyloidosis in 2020.
Blood. 2020 Dec 3;136(23):2620-2627. doi: 10.1182/blood.2020006913.
4
Refining amyloid complete hematological response: Quantitative serum free light chains superior to ratio.
Am J Hematol. 2020 Nov;95(11):1280-1287. doi: 10.1002/ajh.25940. Epub 2020 Aug 24.
5
Systemic Amyloidosis Recognition, Prognosis, and Therapy: A Systematic Review.
JAMA. 2020 Jul 7;324(1):79-89. doi: 10.1001/jama.2020.5493.
6
The utility of repeat kidney biopsy in systemic immunoglobulin light chain amyloidosis.
Amyloid. 2020 Mar;27(1):17-24. doi: 10.1080/13506129.2019.1672650. Epub 2019 Oct 9.
9
AL amyloidosis: advances in diagnostics and treatment.
Nephrol Dial Transplant. 2019 Sep 1;34(9):1460-1466. doi: 10.1093/ndt/gfy291.
10
Depth of organ response in AL amyloidosis is associated with improved survival: grading the organ response criteria.
Leukemia. 2018 Oct;32(10):2240-2249. doi: 10.1038/s41375-018-0060-x. Epub 2018 Feb 20.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验