Plasma Cell Dyscrasia Causing Light Chain Tubulopathy Without Fanconi Syndrome

Elliott, Matthew R.; Cortese, Cherise; Moreno-Aspitia, Alvaro; Dwyer, Jamie P.

doi:10.1053/j.ajkd.2009.08.010

Cited by 22 publications

(19 citation statements)

References 21 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: 96%

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: 96%

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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“…[4][5][6][7][8]15,[17][18][19][20][21][22][23][24][25][26][27][28][29][30][31] In these cases, 53/56 showed the presence of intracytoplasmic crystals in the proximal tubular cells and 37/40 proved to be of the k subtype. The three previous cases without crystal formation were all reported by Kapur et al 5 in one paper.…”

Section: Discussionmentioning

confidence: 99%

The morphologic spectrum and clinical significance of light chain proximal tubulopathy with and without crystal formation

et al. 2011

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The renal diseases most frequently associated with myeloma include amyloidosis, monoclonal immunoglobulin deposition disease, and cast nephropathy. Less frequently reported is light chain proximal tubulopathy, a disease characterized by j-restricted crystal deposits in the proximal tubule cytoplasm. Light chain proximal tubulopathy without crystal deposition is only loosely related to the typical light chain proximal tubulopathy, and little is known about this entity. A search was performed of the 10 081 native kidney biopsy samples processed by our laboratory over the past 2 years for cases that had light chain restriction limited to the proximal tubule cytoplasm. A total of 10 cases of light chain proximal tubulopathy without crystal deposition were found representing 3.1% of light chain-related diseases. Nine of these 10 showed k-light chain restriction. Only three cases of light chain proximal tubulopathy with crystals were found accounting for 0.9% of light chain-related diseases. Two of these three were j subtype. Plasma cell dyscrasia was unsuspected in seven of the 10 patients with light chain proximal tubulopathy without crystals at the time of renal biopsy. After the biopsy was reported, follow-up was available on 9/10 patients with 9/9 showing a plasma cell dyscrasia including 8/9 with multiple myeloma. We found that light chain proximal tubulopathy without crystal formation, despite being rarely described in the literature, is over three times more common than light chain proximal tubulopathy with crystal formation in our series. And given that it is often associated with previously unrecognized myeloma, it is a critically important diagnosis.

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“…We at first assumed that monoclonal BJP-j was the real offender in inducing FS, but careful pathohistological observations did not reveal any evidence of light chain renal involvement. In fact, 'crystalline-like structures' in cytoplasm of proximal tubules are not always detected in cases of monoclonal gammopathy accompanied by 'light chain-induced tubulopathy (including FS)' [13,14], conversely, FS is not always occurred even 'crystalline-like structures' were detected [15], therefore, we cannot conclude simply relying on only pathohistological information. However, steady and gradual reduction of both total urinary protein (including albumin) and M-peak fraction were observed, which strongly support the notion that FS might have been maintained by adefovir administration.…”

Section: Discussionmentioning

confidence: 86%

Acquired Fanconi syndrome due to long-term adefovir administration in a patient with IgG-kappa monoclonal gammopathy and kappa Bence-Jones protein

2014

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A 77-year-old man was admitted to our hospital for a right femoral neck fracture. He had been prescribed lamivudine for chronic hepatitis B infection for 11 years, and adefovir was added 5 years ago. After hospitalization, a right femoral head prosthesis was performed successfully, but an unknown hypokalemia was revealed. Hypophosphatemia, hypouricemia, glucosuria, and panaminoaciduria were also revealed, and multiple microfractures were detected by bone scintigraphy. We diagnosed him as 'osteomalacia associated with Fanconi syndrome,' which was likely due to the adefovir. Moreover, a monoclonal IgG-kappa and a kappa Bence-Jones protein were detected in his serum and urine, respectively. We switched from adefovir plus lamivudine to entecavir and started calcitriol. His excessive urinary b2-microglobulin excretion and glucosuria had decreased dramatically at 10 weeks after the modification of drugs; those of the phosphate, uric acid and total protein, however, continued. Renal biopsy specimens obtained at 10 weeks after discontinuation of adefovir revealed focal tubular atrophic changes with/without inflammatory cells, which were predominantly observed next to glomeruli. Kappa-dominant staining was not observed in either glomeruli or tubules with immunostaining by the enzyme-labeled antibody method. Electron microscopy revealed neither crystalline structures in the cytoplasm of proximal tubules nor electron-dense deposits. Because of the remarkable proportional reduction of other urinary protein fractions, urinary M-peak appeared 26 weeks after discontinuation of adefovir, but the net amounts of the fraction decreased gradually.

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Plasma Cell Dyscrasia Causing Light Chain Tubulopathy Without Fanconi Syndrome

Cited by 22 publications

References 21 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

The morphologic spectrum and clinical significance of light chain proximal tubulopathy with and without crystal formation

Acquired Fanconi syndrome due to long-term adefovir administration in a patient with IgG-kappa monoclonal gammopathy and kappa Bence-Jones protein

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