Renal Light Chain Deposition Associated with the Formation of Intracellular Crystalline Inclusion Bodies in Podocytes: A Rare Case Report

Wang, Yuanda; Dong, Zheyi; Zhang, Xueguang; Zhang, Wei; Yin, Zhimin; Qiu, Qiang; Chen, Xiangmei

doi:10.2169/internalmedicine.55.4443

Cited by 7 publications

(7 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Crystalline podocytopathy is extremely rare, with only 11 cases reported in literature to the best of our knowledge. These cases showed features of significant podocyte injury including nephrotic or subnephrotic glomerular proteinuria and FSGS on renal biopsy [7]. The reported cases showed diverse locations of crystal deposition such as podocytes, tubular epithelial cells, mesangial cells, interstitial histiocytes which were similar with our case.…”

Section: Discussionsupporting

confidence: 86%

Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma

Lee

Park

et al. 2016

Kidney Research and Clinical Practice

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 86%

Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma

Lee

Park

et al. 2016

Kidney Research and Clinical Practice

View full text Add to dashboard Cite

show abstract

“…To date, only 17 cases in native kidneys have been reported in the English literature. Of these, 12 patients had multiple myelomas 7 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 and only 5 had MGRS 22 , 23 , 24 , 25 , 26 ( Table S1 ). Interestingly, all cases (this case included) were associated with IgGκ serum paraprotein.…”

Section: Discussionmentioning

confidence: 99%

Crystal-Induced Podocytopathy Producing Collapsing Focal Segmental Glomerulosclerosis in Monoclonal Gammopathy of Renal Significance: A Case Report

et al. 2021

View full text Add to dashboard Cite

This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

show abstract

“…To the best of our knowledge, only 11 cases of crystalline inclusion within the podocytes associated with plasma cell dyscrasia have been reported. [4,5–15] Of these, nine had multiple myeloma, [5,6,8,9,11–15] and two MGUS. [7,10] Interestingly, all cases had IgG-κ M-protein, with only one case exceptionally manifesting clinical manifestations of Fanconi syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…[3,4] Interestingly, light chain crystalline deposits can be formed within the podocytes. [5–15] In such cases, not Fanconi syndrome but proteinuria, nephrotic syndrome, or renal insufficiency are the main clinical manifestations. However, only very few case reports about the crystalline deposits within the podocytes are available, and the nature of the pathogenic monoclonal light chain implicated in these cases is still unknown.…”

Section: Introductionmentioning

confidence: 99%

A case report of crystalline light chain inclusion-associated kidney disease affecting podocytes but without Fanconi syndrome

et al. 2019

View full text Add to dashboard Cite

Rationale: Crystalline light chain inclusion-associated kidney disease affects mainly tubular epithelial cells and is often clinically manifested as Fanconi syndrome. However, only very few case reports about the crystalline deposits within the podocytes are available, and the nature of the pathogenic monoclonal light chain implicated in these cases is still unknown. We report a case of crystalline inclusion-associated kidney disease manifested as crystalline podocytopathy in which we identified the complete structure of the pathogenic monoclonal light chain as belonging to the germ-line gene of Vκ1-39. Patient concerns: We describe a 65-year-old woman with crystalline light chain inclusion-associated kidney disease showing mild proteinuria and renal insufficiency with monoclonal gammopathy of undetermined significance without Fanconi syndrome. She had crystalline inclusions mainly within podocytes, tubular epithelial cells and histiocytes in the kidney. Light microscopy showed vacuolation of podocytes and tubular epithelial cells, while eosin negative pale needle-like crystals were present within these cells. Electron microscopy showed accumulation of club-like crystals with high electron density in podocytes, proximal tubular epithelial cells and interstitial histiocytes. Clonal analysis revealed that a pathogenic monoclonal light chain was derived from germline gene, Vκ1-39. Diagnoses: The diagnosis of crystalline light chain inclusion-associated kidney disease was made. Interventions and outcomes: Bortezomib and dexamethasone were started and her renal function improved to eGFR 36 mL/min/1.73 m 2 after 9 courses of therapy. Lessons: Patients with light chain crystalline podocytopathy may have a similar pathogenic monoclonal light chain derived from the same germline gene, Vκ1–39, to that of patients with light chain proximal tubulopathy.

show abstract

Renal Light Chain Deposition Associated with the Formation of Intracellular Crystalline Inclusion Bodies in Podocytes: A Rare Case Report

Cited by 7 publications

References 18 publications

Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma

Crystalline podocytopathy and tubulopathy without overt glomerular proteinuria in a patient with multiple myeloma

Crystal-Induced Podocytopathy Producing Collapsing Focal Segmental Glomerulosclerosis in Monoclonal Gammopathy of Renal Significance: A Case Report

A case report of crystalline light chain inclusion-associated kidney disease affecting podocytes but without Fanconi syndrome

Contact Info

Product

Resources

About