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Article Abstract
A 42-year-old man with smoldering immunoglobulin G kappa multiple myeloma showed a heavy proteinuria composed of free light chain, prompting performance of a kidney biopsy. Electron microscopy revealed numerous rhomboid-shaped crystals labelled by the anti-kappa in immunogold, notably in the cytoplasm of podocytes, establishing the diagnosis of crystalline podocytopathy. This case illustrates a rare form of monoclonal gammopathy of renal significance, and highlights the key role of electron microscopy and immunogold to better elucidate the location and composition of crystals.
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| http://dx.doi.org/10.1093/ckj/sfab197 | DOI Listing |
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Light chain crystalline podocytopathy caused by electron-lucent crystals - a diagnostic challenge: a case report.
Ultrastruct Pathol
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UC Davis Comprehensive Cancer Center, Sacramento and Veterans Administration Health Care Center, Mather, CA, USA.
We report here a challenging diagnosis of light chain crystalline podocytopathy in a patient with sub-nephrotic proteinuria, microscopic hematuria, glucosuria and elevated free kappa light chains but without any initial clinical evidence of frank multiple myeloma. We also discuss the diagnostic challenges of this case which include rather unique and seemingly innocuous light microscopic morphology of the glomeruli and the critical roles of paraffin immunofluorescence and need for electron microscopy to confirm the diagnosis. The kappa restriction of the crystals within the podocytes was confirmed only by paraffin immunofluorescence (it went undetected by routine immunofluorescence) and electron microscopy of the glomeruli showed the podocytopathy to be caused by electron-lucent crystals which is a rare variant of light chain crystals, with electron-dense crystals being the predominant variant of light chain crystals.
View Article and Find Full Text PDFMonoclonal immunoglobulin crystalline nephropathies.
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Monoclonal Ig crystalline nephropathies are rare lesions resulting from precipitation of monoclonal Igs in the kidney as crystalline inclusions. They can be categorized into lesions with predominant intracellular crystals (light chain [LC] proximal tubulopathy, LC crystal-storing histiocytosis, and LC crystalline podocytopathy) and lesions with predominant extracellular crystals (crystalglobulin-induced nephropathy and crystalline variant of LC cast nephropathy). The majority of these lesions are associated with low tumor burden lymphoproliferative disorders, with the exception of crystalline variant of LC cast nephropathy.
View Article and Find Full Text PDFCombined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature review.
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Department of Nephrology, Peking University People's Hospital, Beijing, China.
Background: Crystal-storing histiocytosis (CSH), light chain proximal tubulopathy (LCPT), and light chain crystalline podocytopathy (LCCP) are rare complications of multiple myeloma (MM) or monoclonal gammopathy of renal significance, and their diagnoses are challenging.
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Pathological characteristics of light chain crystalline podocytopathy.
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Department of Pathology and Cell Biology, Columbia University Medical Center, New York, New York, USA. Electronic address:
Monoclonal immunoglobulin light chain (LC) crystalline inclusions within podocytes are rare, poorly characterized entities. To provide more insight, we now present the first clinicopathologic series of LC crystalline podocytopathy (LCCP) encompassing 25 patients (68% male, median age 56 years). Most (80%) patients presented with proteinuria and chronic kidney disease, with nephrotic syndrome in 28%.
View Article and Find Full Text PDFCrystalline podocytopathy and tubulopathy linked to kappa light chain deposits in a context of smoldering multiple myeloma.
Clin Kidney J
February 2022
Unité de Biologie cellulaire et microscopie électronique, hôpital Bretonneau CHRU de Tours, Tours, France.
A 42-year-old man with smoldering immunoglobulin G kappa multiple myeloma showed a heavy proteinuria composed of free light chain, prompting performance of a kidney biopsy. Electron microscopy revealed numerous rhomboid-shaped crystals labelled by the anti-kappa in immunogold, notably in the cytoplasm of podocytes, establishing the diagnosis of crystalline podocytopathy. This case illustrates a rare form of monoclonal gammopathy of renal significance, and highlights the key role of electron microscopy and immunogold to better elucidate the location and composition of crystals.
View Article and Find Full Text PDF