Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma
Crystalline nephropathy is a rare yet well-known condition associated with multiple myeloma and other light chain–secreting disorders. Paraproteins that are resistant to proteolysis crystallize within proximal tubular cells and cause light-chain proximal tubulopathy, which presents clinically as Fan...
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| Format: | Article |
| Language: | English |
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The Korean Society of Nephrology
2016-12-01
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| Series: | Kidney Research and Clinical Practice |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2211913216300390 |
| _version_ | 1818190754654191616 |
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| author | Eun Jeong Lee Su Yeon Lee So Young Park Yonjin Kim Jae Shin Choi Mi Jeoung Kim Ji Hyeon Park Jung Eun Lee Ghee Young Kwon Yoon-Goo Kim |
| author_facet | Eun Jeong Lee Su Yeon Lee So Young Park Yonjin Kim Jae Shin Choi Mi Jeoung Kim Ji Hyeon Park Jung Eun Lee Ghee Young Kwon Yoon-Goo Kim |
| author_sort | Eun Jeong Lee |
| collection | DOAJ |
| description | Crystalline nephropathy is a rare yet well-known condition associated with multiple myeloma and other light chain–secreting disorders. Paraproteins that are resistant to proteolysis crystallize within proximal tubular cells and cause light-chain proximal tubulopathy, which presents clinically as Fanconi syndrome. Podocytes are rarely affected, and the crystalline inclusions within podocytes are typically precipitated, yielding significant glomerular proteinuria. Here we report a case of extensive crystalline inclusions primarily within podocytes and proximal tubules that presented only with Fanconi syndrome and renal insufficiency. Despite the presence of extensive crystalline inclusions in podocytes and diffuse foot process effacement, the patient had no clinical evidence suggestive of podocyte injury. |
| first_indexed | 2024-12-12T00:03:45Z |
| format | Article |
| id | doaj.art-3b44929fae9b4766bec8b573e2b6f2eb |
| institution | Directory Open Access Journal |
| issn | 2211-9132 |
| language | English |
| last_indexed | 2024-12-12T00:03:45Z |
| publishDate | 2016-12-01 |
| publisher | The Korean Society of Nephrology |
| record_format | Article |
| series | Kidney Research and Clinical Practice |
| spelling | doaj.art-3b44929fae9b4766bec8b573e2b6f2eb2022-12-22T00:45:10ZengThe Korean Society of NephrologyKidney Research and Clinical Practice2211-91322016-12-0135425926210.1016/j.krcp.2016.06.001Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myelomaEun Jeong Lee0Su Yeon Lee1So Young Park2Yonjin Kim3Jae Shin Choi4Mi Jeoung Kim5Ji Hyeon Park6Jung Eun Lee7Ghee Young Kwon8Yoon-Goo Kim9Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, KoreaGraduate School of Medicine, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Nephrology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Nephrology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, KoreaDepartment of Nephrology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, KoreaCrystalline nephropathy is a rare yet well-known condition associated with multiple myeloma and other light chain–secreting disorders. Paraproteins that are resistant to proteolysis crystallize within proximal tubular cells and cause light-chain proximal tubulopathy, which presents clinically as Fanconi syndrome. Podocytes are rarely affected, and the crystalline inclusions within podocytes are typically precipitated, yielding significant glomerular proteinuria. Here we report a case of extensive crystalline inclusions primarily within podocytes and proximal tubules that presented only with Fanconi syndrome and renal insufficiency. Despite the presence of extensive crystalline inclusions in podocytes and diffuse foot process effacement, the patient had no clinical evidence suggestive of podocyte injury.http://www.sciencedirect.com/science/article/pii/S2211913216300390Fanconi syndromeMultiple myelomaNephropathy |
| spellingShingle | Eun Jeong Lee Su Yeon Lee So Young Park Yonjin Kim Jae Shin Choi Mi Jeoung Kim Ji Hyeon Park Jung Eun Lee Ghee Young Kwon Yoon-Goo Kim Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma Kidney Research and Clinical Practice Fanconi syndrome Multiple myeloma Nephropathy |
| title | Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma |
| title_full | Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma |
| title_fullStr | Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma |
| title_full_unstemmed | Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma |
| title_short | Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma |
| title_sort | crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma |
| topic | Fanconi syndrome Multiple myeloma Nephropathy |
| url | http://www.sciencedirect.com/science/article/pii/S2211913216300390 |
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