Wu HT, Wen YB, Ye W, Liu BY, Shen KN, Gao RT, Li MX. Underlying IgM heavy chain amyloidosis in treatment-refractory IgA nephropathy: A case report. World J Clin Cases 2019; 7(19): 3055-3061 [PMID: 31624754 DOI: 10.12998/wjcc.v7.i19.3055]
Corresponding Author of This Article
Rui-Tong Gao, MD, Associate Professor, Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Dongcheng District, Beijing 100730, China. gaoruitong@gmail.com
Research Domain of This Article
Medicine, Research & Experimental
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Oct 6, 2019; 7(19): 3055-3061 Published online Oct 6, 2019. doi: 10.12998/wjcc.v7.i19.3055
Underlying IgM heavy chain amyloidosis in treatment-refractory IgA nephropathy: A case report
Hai-Ting Wu, Yu-Bing Wen, Wei Ye, Bing-Yan Liu, Kai-Ni Shen, Rui-Tong Gao, Ming-Xi Li
Hai-Ting Wu, Yu-Bing Wen, Wei Ye, Bing-Yan Liu, Rui-Tong Gao, Ming-Xi Li, Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
Kai-Ni Shen, Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
Author contributions: Wu HT and Liu BY managed the patient and collected the data; Wen YB and Ye W reported the pathological findings; Shen KN performed laser microdissection/mass spectrometry; Gao RT and Li MX were involved in revising the manuscript; All authors read and approved the final manuscript.
Informed consent statement: Written informed consent was obtained.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Rui-Tong Gao, MD, Associate Professor, Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Dongcheng District, Beijing 100730, China. gaoruitong@gmail.com
Telephone: +86-10-69155058 Fax: +86-10-69155058
Received: April 13, 2019 Peer-review started: April 15, 2019 First decision: August 1, 2019 Revised: August 14, 2019 Accepted: August 27, 2019 Article in press: August 27, 2019 Published online: October 6, 2019 Processing time: 170 Days and 0.3 Hours
Abstract
BACKGROUND
Monoclonal immunoglobulin can cause renal damage, with a wide spectrum of pathological changes and clinical manifestations without hematological evidence of malignancy. These disorders can be missed, especially when combined with other kidney diseases.
CASE SUMMARY
A 61-year-old woman presented with moderate proteinuria with normal renal function. She was diagnosed with IgA nephropathy combined with monoclonal gammopathy of undetermined significance after the first renal biopsy. Although having received immunosuppressive treatment for 3 years, the patient developed nephrotic syndrome. Repeated renal biopsy and laser microdissection/mass spectrometry analysis confirmed heavy chain amyloidosis. After nine cycles of bortezomib, cyclophosphamide and dexamethasone, she achieved very good partial hematological and kidney responses.
CONCLUSION
Renal injury should be monitored closely in monoclonal gammopathy patients without obvious hematological malignancy, especially in patients with other pre-existing renal diseases.
Core tip: Monoclonal immunoglobulin can cause renal injury without hematological evidence of malignancy. These disorders can be missed, especially when combined with other kidney diseases. We present a patient initially diagnosed with IgA nephropathy and monoclonal gammopathy of undetermined significance (MGUS). Atypical characteristics and treatment refractoriness of IgA nephropathy led to a second renal biopsy. Amyloidosis was revealed, and laser microdissection/mass spectrometry was used for typing. The patient improved well after therapy containing bortezomib. Renal involvement should be monitored closely in patients with MGUS, especially in those with pre-existing kidney diseases.
