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Two kinds of rare light chain cast nephropathy caused by multiple myeloma: case reports and literature review
BACKGROUND: Light chain cast nephropathy (LCCN) is the most common renal disease caused by multiple myeloma (MM). In addition to ordinary light chain protein casts, there are a few rare casts with unique shapes, including light chain amyloid casts (LCAC) and light chain crystal casts (LCCC). CASE PR...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7845023/ https://www.ncbi.nlm.nih.gov/pubmed/33509125 http://dx.doi.org/10.1186/s12882-021-02250-z |
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author | Sun, Li-Jun Dong, Hong-Rui Xu, Xiao-Yi Wang, Guo-Qin Cheng, Hong Chen, Yi-Pu |
author_facet | Sun, Li-Jun Dong, Hong-Rui Xu, Xiao-Yi Wang, Guo-Qin Cheng, Hong Chen, Yi-Pu |
author_sort | Sun, Li-Jun |
collection | PubMed |
description | BACKGROUND: Light chain cast nephropathy (LCCN) is the most common renal disease caused by multiple myeloma (MM). In addition to ordinary light chain protein casts, there are a few rare casts with unique shapes, including light chain amyloid casts (LCAC) and light chain crystal casts (LCCC). CASE PRESENTATIONS: Here, we report two patients. Patient 1 is a 72-year-old man who was clinically diagnosed with MM and acute kidney injury (AKI). Pathological examination of a renal biopsy revealed that there were many amyloid casts in the distal tubules that had a lightly-stained central area and a deeply-stained burr-like edge. The marginal zone of the cast was positive for Congo red staining and contained numerous amyloid fibers, as observed by electron microscopy. No systemic amyloidosis was found. The patient received 4 courses of bortezomib-based chemotherapy, and then, his MM achieved partial remission. Patient 2 is a 57-year-old man who was also clinically diagnosed with MM and AKI. Pathological examination of a renal biopsy showed that there were many crystalline casts in the distal tubules that were fully or partially composed of crystals with different shapes, including rhomboid, needle, triangle, rectangle and other geometric shapes. Congo red staining was negative. Crystals were also detected in the urine of this patient. After 9 courses of treatment with a bortezomib-based regimen, his MM obtained complete remission and his renal function returned to normal. CONCLUSIONS: LCAC and LCCC nephropathy caused by MM are two rare types of LCCN, and both have their own unique morphological manifestations. LCAC nephropathy may not be accompanied by systemic amyloidosis. The diagnosis of these two unique LCCNs must rely on renal biopsy pathology, and the discovery of urine crystals is of great significance for indicating LCCC nephropathy. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12882-021-02250-z. |
format | Online Article Text |
id | pubmed-7845023 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-78450232021-02-01 Two kinds of rare light chain cast nephropathy caused by multiple myeloma: case reports and literature review Sun, Li-Jun Dong, Hong-Rui Xu, Xiao-Yi Wang, Guo-Qin Cheng, Hong Chen, Yi-Pu BMC Nephrol Case Report BACKGROUND: Light chain cast nephropathy (LCCN) is the most common renal disease caused by multiple myeloma (MM). In addition to ordinary light chain protein casts, there are a few rare casts with unique shapes, including light chain amyloid casts (LCAC) and light chain crystal casts (LCCC). CASE PRESENTATIONS: Here, we report two patients. Patient 1 is a 72-year-old man who was clinically diagnosed with MM and acute kidney injury (AKI). Pathological examination of a renal biopsy revealed that there were many amyloid casts in the distal tubules that had a lightly-stained central area and a deeply-stained burr-like edge. The marginal zone of the cast was positive for Congo red staining and contained numerous amyloid fibers, as observed by electron microscopy. No systemic amyloidosis was found. The patient received 4 courses of bortezomib-based chemotherapy, and then, his MM achieved partial remission. Patient 2 is a 57-year-old man who was also clinically diagnosed with MM and AKI. Pathological examination of a renal biopsy showed that there were many crystalline casts in the distal tubules that were fully or partially composed of crystals with different shapes, including rhomboid, needle, triangle, rectangle and other geometric shapes. Congo red staining was negative. Crystals were also detected in the urine of this patient. After 9 courses of treatment with a bortezomib-based regimen, his MM obtained complete remission and his renal function returned to normal. CONCLUSIONS: LCAC and LCCC nephropathy caused by MM are two rare types of LCCN, and both have their own unique morphological manifestations. LCAC nephropathy may not be accompanied by systemic amyloidosis. The diagnosis of these two unique LCCNs must rely on renal biopsy pathology, and the discovery of urine crystals is of great significance for indicating LCCC nephropathy. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12882-021-02250-z. BioMed Central 2021-01-28 /pmc/articles/PMC7845023/ /pubmed/33509125 http://dx.doi.org/10.1186/s12882-021-02250-z Text en © The Author(s) 2021 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Sun, Li-Jun Dong, Hong-Rui Xu, Xiao-Yi Wang, Guo-Qin Cheng, Hong Chen, Yi-Pu Two kinds of rare light chain cast nephropathy caused by multiple myeloma: case reports and literature review |
title | Two kinds of rare light chain cast nephropathy caused by multiple myeloma: case reports and literature review |
title_full | Two kinds of rare light chain cast nephropathy caused by multiple myeloma: case reports and literature review |
title_fullStr | Two kinds of rare light chain cast nephropathy caused by multiple myeloma: case reports and literature review |
title_full_unstemmed | Two kinds of rare light chain cast nephropathy caused by multiple myeloma: case reports and literature review |
title_short | Two kinds of rare light chain cast nephropathy caused by multiple myeloma: case reports and literature review |
title_sort | two kinds of rare light chain cast nephropathy caused by multiple myeloma: case reports and literature review |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7845023/ https://www.ncbi.nlm.nih.gov/pubmed/33509125 http://dx.doi.org/10.1186/s12882-021-02250-z |
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