Non-crystalline light chain proximal tubulopathy, a morphologically protean entity.

Andreas Kousios,Sarah Blakey,Linda Moran,Maria Atta,Rawya Charif,Neill Duncan,Andrew Smith,Frederick W K Tam,Jeremy B Levy,Aristeidis Chaidos,Candice Roufosse

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association（2023）

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Abstract

The non-crystalline variant may go unrecognised because of its subtle histopathological features and requires EM to distinguish it from "excessive LC resorption without tubular injury". Clone-directed treatment with good haematological response improves renal outcomes in both variants but limited data exist in MGRS. Multi-centre prospective studies are needed to better define the clinico-pathological characteristics associated with poor outcomes and optimize treatment strategies in patients with MGRS.

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Key words

chronic kidney disease, Fanconi syndrome, monoclonal gammopathy of renal significance, multiple myeloma, proximal tubular cells

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