Light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits is associated with a high detection rate of the pathogenic plasma cell clone

2020 ◽  
Vol 97 (3) ◽  
pp. 589-601 ◽  
Author(s):  
Samih H. Nasr ◽  
Christopher P. Larsen ◽  
Christophe Sirac ◽  
Jason D. Theis ◽  
Camille Domenger ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Light Chain ◽  
Detection Rate ◽  
Cell Clone ◽  
Proliferative Glomerulonephritis ◽  
Monoclonal Immunoglobulin ◽  
High Detection Rate ◽  
High Detection ◽  
Immunoglobulin Deposits

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits: a nephrologist perspective

2019 ◽  
Author(s):  
Frank Bridoux ◽  
Vincent Javaugue ◽  
Samih H Nasr ◽  
Nelson Leung
Keyword(s):  
Cell Clone ◽  
Early Recurrence ◽  
Proliferative Glomerulonephritis ◽  
Monoclonal Immunoglobulin ◽  
Electron Microscopic ◽  
Nephritic Syndrome ◽  
Microscopic Studies ◽  
Immunoglobulin Deposits ◽  
Stage Renal Disease ◽  
End Stage

AbstractProliferative glomerulonephritis (GN) with monoclonal immunoglobulin deposits (PGNMIDs) is a recently described entity among the spectrum of monoclonal gammopathy of renal significance (MGRS). The disease is renal limited and manifests with chronic glomerular disease, altered renal function and albuminuria, sometimes in the nephrotic range. Acute nephritic syndrome is rare. PGNMID occurs mostly in the sixth decade, but it may affect young adults. Histologically, PGNMID is characterized predominantly by membranoproliferative GN and less frequently by diffuse endocapillary GN, mesangioproliferative GN or atypical membranous GN. Immunofluorescence and electron microscopic studies are the cornerstone of diagnosis, showing granular deposits involving glomeruli only, and composed of monotypic immunoglobulin G (IgG), with a single heavy chain subclass (most commonly IgG3) and light chain (LC) restriction (usually κ), admixed with complement deposits. PGNMID variants with monotypic LC-only, IgA or IgM deposits are uncommon. Ultrastructurally, deposits are amorphous with predominant subendothelial and mesangial distribution. PGNMID should be distinguished from type 1 cryoglobulinemic GN and immunotactoid GN, which share some common pathological features. Contrary to other MGRS lesions, the rate of detection of the nephrotoxic monoclonal Ig in the serum or urine, and of an abnormal bone marrow B-cell clone, is only ∼30%. Renal prognosis is poor, with progression to end-stage renal disease in 25% of patients within 30 months and frequent early recurrence on the renal allograft. The pathophysiology of PGNMID is unclear and its treatment remains challenging. However, recent studies indicate that clone-targeted chemotherapy may significantly improve renal outcomes, opening future perspectives for the management of this rare disease.

scholarly journals Nephrotic Syndrome Secondary to Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits of Lambda Light Chain

2014 ◽  
Vol 2014 ◽  
pp. 1-6
Author(s):  
Seongseok Yun ◽  
Beth L. Braunhut ◽  
Courtney N. Walker ◽  
Waheed Bhati ◽  
Amy N. Sussman ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Light Chain ◽  
Hematologic Malignancies ◽  
Proliferative Glomerulonephritis ◽  
Lambda Light Chain ◽  
Monoclonal Immunoglobulin ◽  
Subendothelial Space ◽  
History Of ◽  
Immunoglobulin Deposits ◽  
Refractory Nephrotic Syndrome

We describe a rare case of a 46-year-old woman with history of refractory nephrotic syndrome and hypertension who presented with worsening proteinuria and kidney function. Work-up for both autoimmune and infectious diseases and hematologic malignancies including multiple myeloma were negative. Kidney biopsy demonstrated glomerular sclerotic change with lambda light chain deposits in the subendothelial space, which is consistent with proliferative glomerulonephritis with monoclonal immunoglobulin deposit (PGNMID). The patient was treated with bortezomib and dexamethasone without clinical improvement and eventually became hemodialysis dependent.

Infiltrative Nonamyloidotic Monoclonal Immunoglobulin Light Chain Cardiomyopathy: An Underappreciated Manifestation of Plasma Cell Dyscrasias

Cardiology ◽  
2000 ◽  
Vol 93 (4) ◽  
pp. 220-228 ◽  
Author(s):  
Joel N. Buxbaum ◽  
Elizabeth M. Genega ◽  
Piotr Lazowski ◽  
Asok Kumar ◽  
Paul A. Tunick ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Light Chain ◽  
Immunoglobulin Light Chain ◽  
Monoclonal Immunoglobulin ◽  
Plasma Cell Dyscrasias

A Comparative Review of Various Machine Learning Approaches for Improving the Performance of Stego Anomaly Detection

2018 ◽  
pp. 351-371 ◽  
Author(s):  
Hemalatha Jeyaprakash ◽  
KavithaDevi M. K. ◽  
Geetha S.
Keyword(s):  
Machine Learning ◽  
Anomaly Detection ◽  
Detection Rate ◽  
High Dimensional ◽  
Learning Approaches ◽  
Learning Tools ◽  
High Detection Rate ◽  
Comparative Review ◽  
High Detection

In recent years, steganalyzers are intelligently detecting the stego images with high detection rate using high dimensional cover representation. And so the steganographers are working towards this issue to protect the cover element dependency and to protect the detection of hiding secret messages. Any steganalysis algorithm may achieve its success in two ways: 1) extracting the most sensitive features to expose the footprints of message hiding; 2) designing or building an effective classifier engine to favorably detect the stego images through learning all the stego sensitive features. In this chapter, the authors improve the stego anomaly detection using the second approach. This chapter presents a comparative review of application of the machine learning tools for steganalysis problem and recommends the best classifier that produces a superior detection rate.

scholarly journals Conventional Therapy for Amyloid Light-Chain Amyloidosis

2020 ◽  
Vol 143 (4) ◽  
pp. 365-372
Author(s):  
Paolo Milani ◽  
Giovanni Palladini
Keyword(s):  
Stem Cell ◽  
Plasma Cell ◽  
Light Chain ◽  
Cell Clone ◽  
Alkylating Agents ◽  
Proteasome Inhibitors ◽  
Response Rates ◽  
Al Amyloidosis ◽  
Cell Transplant ◽  
Conventional Chemotherapy

The vast majority of patients with light-chain (AL) amyloidosis are not eligible for stem cell transplant and are treated with conventional chemotherapy. Conventional regimens are based on various combinations of dexamethasone, alkylating agents, proteasome inhibitors, and immunomodulatory drugs. The choice of these regimens requires a careful risk stratification, based on the extent of amyloid organ involvement, comorbidities, and the characteristics of the amyloidogenic plasma cell clone. Most patients are treated upfront with bortezomib and dexamethasone combined with cyclophosphamide or melphalan. Cyclophosphamide does not compromise stem cell mobilization and harvest and is more manageable in renal failure. Melphalan can overcome the effect of t(11;14), which is associated with lower response rates and shorter survival in subjects treated with bortezomib and dexamethasone, or in combination with cyclophosphamide. Lenalidomide and pomalidomide are the mainstay of rescue treatment. They are effective in patients exposed to bortezomib, dexamethasone, and alkylators, but deep hematologic responses are rare. Ixazomib, alone or in combination with lenalidomide, increases the rate of complete responses in relapsed/refractory patients. Conventional chemotherapy regimens will represent the backbone for future combinations, particularly with anti-plasma-cell immunotherapy, that will further improve response rates and outcomes.

A Approach to Change Detection for HR Image

2014 ◽  
Vol 971-973 ◽  
pp. 1449-1453
Author(s):  
Zuo Wei Huang ◽  
Shu Guang Wu ◽  
Tao Xin Zhang
Keyword(s):  
Change Detection ◽  
Detection Rate ◽  
False Detection Rate ◽  
False Detection ◽  
High Detection Rate ◽  
High Detection ◽  
Self Adaptation ◽  
Ica Algorithm ◽  
Different Characteristics ◽  
Better Than

Hyperspectral remote sensing is the multi-dimensional information obtaining technology,which combines target detection and spectral imaging technology together, In order to accord with the condition of hyperspectral imagery，the paper developed an optimized ICA algorithm for change detection to describe the statistical distribution of the data. By processing these abundance maps, change of different classes of objects can be obtained..A approach is capable of self-adaptation, and can be applied to hyperspectral images with different characteristics. Experiment results demonstrate that the ICA-based hyperspectral change detection performs better than other traditional methods with a high detection rate and a low false detection rate.

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