The characteristics of patients with kidney light chain deposition disease concurrent with light chain amyloidosis

Said, Samar M.; Rocha, Alejandro Best; Valeri, Anthony M.; Paueksakon, Paisit; Dasari, Surendra; Theis, Jason D.; Vrana, Julie A.; Obadina, Modupe O.; Saghafi, Darius; Alexander, Mariam P.; Sethi, Sanjeev; Larsen, Christopher P.; Joly, Florent; Dispenzieri, Angela; Bridoux, Frank; Sirac, Christophe; Leung, Nelson; Fogo, Agnes B.; McPhail, Ellen D.; Nasr, Samih H.

doi:10.1016/j.kint.2021.10.019

Cited by 7 publications

(8 citation statements)

References 40 publications

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“…The largest study to date on combined renal AL amyloidosis and MIDD revealed that deposits had the same LC isotype and variable domain subgroup. The authors endorse the hypothesis that subclones stemming from LC rearrangements with variants in one of the CDRs may drive the phenotypic heterogeneity [6]. A similar pathomechanism may be causing the two structurally different deposits seen in our patient although other organ‐specific factors such as different pH value or chaperones may also play a role.…”

Section: Figuresupporting

confidence: 74%

“…Hypotheses on the coexistence of AL amyloidosis and MIDD include different conformations of the same pathogenic LC, subclonal somatic variants of the original LC gene or the presence of biclonal gammopathy. The largest study to date on combined renal AL amyloidosis and MIDD revealed that deposits had the same LC isotype and variable domain subgroup.The authors endorse the hypothesis that subclones stemming from LC rearrangements with variants in one of the CDRs may drive the phenotypic heterogeneity[6]. A similar pathomechanism may be causing the two structurally different deposits seen in our patient although otherF I G U R E 2 Crystalline inclusions of monoclonal kappa light chains in proximal tubular epithelial cells and bone marrow plasma cells.…”

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confidence: 77%

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Concurrent light chain amyloidosis and proximal tubulopathy: Insights into different aggregation behavior—A case report

Feurstein

Zoller

Schwab

et al. 2022

eJHaem

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Due to differences in the protein folding mechanisms, it is exceedingly rare for amyloid light chain (AL) amyloidosis and monoclonal gammopathy of renal significance (MGRS) to coexist. We herein report the first case of concurrent AL amyloidosis and a subclass of MGRS, light chain proximal tubulopathy (LCPT). The 53‐year‐old female was diagnosed with smoldering myeloma immunoglobulin G kappa and AL amyloidosis with deposits in fat and gastrointestinal tissue. The kidney biopsy did not show amyloid deposits but electron microscopy revealed the presence of LCPT with crystal formation in proximal tubular epithelial cells. This case illustrates the complex pathophysiology of protein deposition in monoclonal gammopathies.

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Section: Figuresupporting

confidence: 74%

supporting

confidence: 77%

Concurrent light chain amyloidosis and proximal tubulopathy: Insights into different aggregation behavior—A case report

Feurstein

Zoller

Schwab

et al. 2022

eJHaem

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“…We reported a rare MIg-associated renal disease in which MIg deposited in both organized and non-organized ultrastructures. According to the pathogenesis reported ( 10 ), MIDD and ITG might resulted from immunoglobulins (MIgs) of different origins with unusual or abnormal structures. The deposition may be the consequence of the acquired defects in podocyte functions relating the clearance of the filtrated and retained immunoglobulin, which created the unique environment for deposition ( 19 , 25 ).…”

Section: Discussionmentioning

confidence: 99%

“…The most common pathologic form is monoclonal immunoglobulin deposition disease (MIDD) coexisting with light chain cast nephropathy (LCCN) ( 4 , 6 – 9 ). However, the combination of two forms of glomerular diseases, especially the co-deposition of organized and non-organized structures was rare ( 3 , 10 ). The presentation of this combination was not simply the add-on, but had unique characteristics.…”

Section: Introductionmentioning

confidence: 99%

Clinicopathological manifestations of coexistent monoclonal immunoglobulin deposition disease and immunotactoid glomerulopathy

Wang

Yan²,

Dong³

et al. 2022

Front. Med.

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Combination of monoclonal immunoglobulin deposition disease (MIDD) and immunotactoid glomerulopathy (ITG) is a rare form of monoclonal immunoglobulin (MIg)-associated renal disease. We retrospectively reviewed the native kidney biopsy specimens at Peking University People’s Hospital from 2011 to 2020. Five patients were diagnosed as MIDD + ITG. Their clinical and pathological characteristics were studied. The typical clinical features were nephritic syndrome and renal dysfunction with prominent anemia, but hematuria was mild. Unlike single MIDD and single ITG, on light microscopy, segmentally distributed mesangial nodular sclerosis on the basis of mesangial matrix hyperplasia was the major lesion. Others including membranoproliferative glomerulonephritis (MPGN)-like lesion, glomerular basement membrane thickness, and mild to moderate mesangial and endothelial proliferations might presented at the same time and in the same glomeruli. On immunofluorescence, MIg, usually monoclonal light chains, deposited along glomerular basement membranes and tubular basement membranes, while the intact MIg or monoclonal heavy chain deposited in the mesangial regions. Corresponding to the depositions on immunofluorescence, punctate “powdery” deposits along glomerular basement membranes and tubular basement membranes under electronic microscopy indicated the presence of MIDD. Microtubular substructures (diameters of 20–50 nm) exhibiting hollow cores arranged in parallel arrays in mesangial regions indicated the presence of ITG. Patients treated with bortezomib-based regimen seemed to have better outcomes. In conclusion, MIDD + ITG is a rare combination form of MIg-associated renal disease. Accurate diagnosis requires the comprehensive pathological investigations.

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“…Light chains can cause more than one type of kidney disease. For example, patients with LA may also have light chain deposition disease (LCDD) with or without light-chain cast nephropathy [ 6 ]. Non-organized, granular deposition along the TBM is well known in LCDD; however, little is known about the frequency, clinical and prognostic importance of amyloid fibril deposition along the TBM in systemic LA.…”

Section: Introductionmentioning

confidence: 99%

Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis?

et al. 2023

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In light chain amyloidosis (LA), the massive glomerular and vascular amyloid deposition leading to interstitial fibrosis/tubular atrophy (IFTA) is thought to be responsible for renal failure. The amyloid deposition in the interstitium and the tubular basement membrane (TBM) has received less attention in the study of LA. We, therefore, collected clinical and laboratory data on patients diagnosed with LA in our Nephrology Department and studied amyloid deposition in the TBM. Twelve LA patients were diagnosed by renal biopsy during a seven-year period. In 4 of the 12, amyloid deposition could also be detected in the TBM. In our first case of a patient with diabetes mellitus, non-amyloid fibrils resembling ‘diabetic fibrillosis’ were also seen by electron microscopy. Despite the double damage, IFTA was negligible, blood vessels were unaffected, and the glomerular deposition was segmental. In the other three cases, significant (>50%) IFTA and a severely reduced estimated glomerular filtration rate were already detected at the time of diagnosis and amyloid deposition was also observed in the blood vessels. These findings indicate the importance of TBM amyloid deposition in the progression of renal disease. This may represent a late-stage presentation of the disease with a heavy LC burden.

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The characteristics of patients with kidney light chain deposition disease concurrent with light chain amyloidosis

Cited by 7 publications

References 40 publications

Concurrent light chain amyloidosis and proximal tubulopathy: Insights into different aggregation behavior—A case report

Concurrent light chain amyloidosis and proximal tubulopathy: Insights into different aggregation behavior—A case report

Clinicopathological manifestations of coexistent monoclonal immunoglobulin deposition disease and immunotactoid glomerulopathy

Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis?

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