Crystalline Inclusions in the Glomerular Podocytes in a Patient With Benign Monoclonal Gammopathy and Focal Segmental Glomerulosclerosis

Matsuyama, Norimasa; Joh, Kensuke; Yamaguchi, Yutaka; Aizawa, Shigeo; Kanai, Tatsuya; Kitajima, T; Sakai, Osamu

doi:10.1016/s0272-6386(12)80141-7

Cited by 30 publications

(21 citation statements)

References 7 publications

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“…In this previous study, the diagnosis of malignant plasma cells could not be established for some patients at the time of renal biopsy, although the patients were diagnosed later in a follow-up study with the gradual emergence of corresponding clinical manifestations (10). In contrast, it is very rare to present large quantities of crystalline inclu- sions deposited in podocytes and only 10 cases have been reported to date (2,5,6,(11)(12)(13)(14)(15)(16)(17) (Table). Therefore, the present case, which was identified via electron microscopy, represents the 11th case reported.…”

Section: Discussionmentioning

confidence: 95%

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

et al. 2016

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We herein report the case of an elderly woman with bone pain and proteinuria as the main clinical manifestations. The patient was diagnosed with the IgG κ type of multiple myeloma. Her renal pathology consisted of widespread κ light chain protein deposition associated with the formation of large quantities of rodlike crystals in podocytes. This phenomenon is very rare. We explored the significance of this crystal formation via a detailed and descriptive analysis and also performed a literature review, thus providing data to increase the available information about this type of disease.

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Section: Discussionmentioning

confidence: 95%

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

et al. 2016

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“…Only a few other cases of LCP have been reported in the literature, all of which presented with varying degrees of kidney insufficiency and proteinuria. Proximal tubulopathy was concurrently identified in all cases . The reported glomerular findings by light microscopy include swollen podocytes with cytoplasmic inclusions, similar to those seen in tubular epithelial cells.…”

Section: Discussionmentioning

confidence: 62%

Light Chain Podocytopathy Mimicking Recurrent Focal Segmental Glomerulosclerosis

Khalighi

Revelo

Abraham

et al. 2017

American Journal of Transplantation

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Kidney injury related to paraproteinemia is common and typically occurs after the fourth decade of life in association with an underlying plasma cell dyscrasia or other lymphoproliferative disease. Kidney transplantation in paraprotein-related kidney disease can be successful in conjunction with treatment of the underlying hematopoietic process; however, when hematologic response to therapy is not achieved, recurrent kidney injury is frequently seen. We describe a young male patient who presented at the age of 23 years with end-stage kidney disease thought to be secondary to focal segmental glomerulosclerosis; this patient ultimately received two kidney allografts. He experienced recurrent proteinuria in both kidneys, with a biopsy from his second allograft showing kappa-restricted crystalline light chain podocytopathy, which was identified in both his native and first allograft kidneys upon retrospective review. Recurrent light chain podocytopathy has not been previously reported but poses a diagnostic challenge as it can mimic focal segmental glomerulosclerosis, particularly in young patients in whom paraprotein-related kidney injury is usually not suspected.

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“…Although the reasons underlying the absence of overt glomerular proteinuria are presently unknown, we speculate that two possibilities. Three cases described nephrotic syndrome accompanying with FSGS and two cases showed collapsing glomerulopathy which resulted from catastrophic injury to podocytes [5], [9], [10]. No FSGS in pathologic findings and no risk factors to collapsing glomerulopathy in this case were related with subnephrotic proteinuria.…”

Section: Discussionmentioning

confidence: 62%

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

Lee

Park

et al. 2016

Kidney Research and Clinical Practice

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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.

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Crystalline Inclusions in the Glomerular Podocytes in a Patient With Benign Monoclonal Gammopathy and Focal Segmental Glomerulosclerosis

Cited by 30 publications

References 7 publications

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

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

Light Chain Podocytopathy Mimicking Recurrent Focal Segmental Glomerulosclerosis

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

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