Clinical and Pathological Characteristics of Non-AL Amyloidosis MGRS: A Single-Center Experience Over 10 Years.

IF 1.6 Q3 UROLOGY & NEPHROLOGY
Canadian Journal of Kidney Health and Disease Pub Date : 2025-02-21 eCollection Date: 2025-01-01 DOI:10.1177/20543581251318830
Chunpeng Nie, Holly Lee, Kim Cheema, Peter Duggan, Sylvia McCulloch, Jason Tay, Paola Neri, Nizar J Bahlis, Victor H Jimenez-Zepeda
{"title":"Clinical and Pathological Characteristics of Non-AL Amyloidosis MGRS: A Single-Center Experience Over 10 Years.","authors":"Chunpeng Nie, Holly Lee, Kim Cheema, Peter Duggan, Sylvia McCulloch, Jason Tay, Paola Neri, Nizar J Bahlis, Victor H Jimenez-Zepeda","doi":"10.1177/20543581251318830","DOIUrl":null,"url":null,"abstract":"<p><strong>Objective: </strong>Monoclonal gammopathy of renal significance (MGRS) is a heterogeneous and relatively recently defined disorder that encompasses many kidney and hematologic pathologies. MGRS remains a rare disease and there is a need for more literature regarding its treatment and outcomes. In this study, we share our center's experience with MGRS including incidence of different kidney pathologies, clone type, kidney and hematologic response, and progression-free survival.</p><p><strong>Methods: </strong>Data from 35 patients diagnosed with MGRS excluding light-chain amyloidosis between 2013 and 2022 at a single Canadian tertiary care center were retrospectively analyzed. All cases required kidney biopsy. Initial treatment included regimens containing bortezomib, rituximab, or cyclosporine, or steroids only. Parameters studied included incidence of different kidney pathologies, clone type, depth of hematologic response, kidney survival (KS), overall survival (OS), and progression-free survival (PFS).</p><p><strong>Results: </strong>Out of 35 patients, there were 10 cases of monoclonal immunoglobulin deposition disease, 8 of proliferative glomerulonephritis with immune deposits, 5 of microtubular immune deposits including immunotactoid and types 1 and 2 cryoglobulinemic nephropathy, 3 of C3 glomerulonephritis, and 9 of other diagnoses. There were 21 cases with a plasma cell clone identified in bone marrow, 2 each of B cell and low-grade lymphoma, 1 atypical T cell clone, and 9 cases without an expanded clone on bone marrow biopsy. A total of 6 patients required kidney replacement therapy and 4 patients died; the median PFS was 59.3 months. Very good partial hematologic response or better was significantly associated with decreased proteinuria but not preserved eGFR. There was a non-significant trend toward better PFS with hematologic response.</p><p><strong>Conclusion: </strong>Our experience confirms that MGRS is a heterogeneous disease and adds to the literature concerning the diagnosis and treatment of MGRS. Successful treatment of the underlying hematologic disorder with targeted therapy is more likely to lead to an improvement in kidney function.</p>","PeriodicalId":9426,"journal":{"name":"Canadian Journal of Kidney Health and Disease","volume":"12 ","pages":"20543581251318830"},"PeriodicalIF":1.6000,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11843701/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Canadian Journal of Kidney Health and Disease","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1177/20543581251318830","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
引用次数: 0

Abstract

Objective: Monoclonal gammopathy of renal significance (MGRS) is a heterogeneous and relatively recently defined disorder that encompasses many kidney and hematologic pathologies. MGRS remains a rare disease and there is a need for more literature regarding its treatment and outcomes. In this study, we share our center's experience with MGRS including incidence of different kidney pathologies, clone type, kidney and hematologic response, and progression-free survival.

Methods: Data from 35 patients diagnosed with MGRS excluding light-chain amyloidosis between 2013 and 2022 at a single Canadian tertiary care center were retrospectively analyzed. All cases required kidney biopsy. Initial treatment included regimens containing bortezomib, rituximab, or cyclosporine, or steroids only. Parameters studied included incidence of different kidney pathologies, clone type, depth of hematologic response, kidney survival (KS), overall survival (OS), and progression-free survival (PFS).

Results: Out of 35 patients, there were 10 cases of monoclonal immunoglobulin deposition disease, 8 of proliferative glomerulonephritis with immune deposits, 5 of microtubular immune deposits including immunotactoid and types 1 and 2 cryoglobulinemic nephropathy, 3 of C3 glomerulonephritis, and 9 of other diagnoses. There were 21 cases with a plasma cell clone identified in bone marrow, 2 each of B cell and low-grade lymphoma, 1 atypical T cell clone, and 9 cases without an expanded clone on bone marrow biopsy. A total of 6 patients required kidney replacement therapy and 4 patients died; the median PFS was 59.3 months. Very good partial hematologic response or better was significantly associated with decreased proteinuria but not preserved eGFR. There was a non-significant trend toward better PFS with hematologic response.

Conclusion: Our experience confirms that MGRS is a heterogeneous disease and adds to the literature concerning the diagnosis and treatment of MGRS. Successful treatment of the underlying hematologic disorder with targeted therapy is more likely to lead to an improvement in kidney function.

求助全文
约1分钟内获得全文 求助全文
来源期刊
CiteScore
3.00
自引率
5.90%
发文量
84
审稿时长
12 weeks
期刊介绍: Canadian Journal of Kidney Health and Disease, the official journal of the Canadian Society of Nephrology, is an open access, peer-reviewed online journal that encourages high quality submissions focused on clinical, translational and health services delivery research in the field of chronic kidney disease, dialysis, kidney transplantation and organ donation. Our mandate is to promote and advocate for kidney health as it impacts national and international communities. Basic science, translational studies and clinical studies will be peer reviewed and processed by an Editorial Board comprised of geographically diverse Canadian and international nephrologists, internists and allied health professionals; this Editorial Board is mandated to ensure highest quality publications.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信