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Light Chain Proximal Tubulopathy MGRS — ESENeph MCQ

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HardTubular DisordersLight Chain Proximal Tubulopathy MGRSESENeph

A patient has Fanconi syndrome and biopsy-proven light-chain proximal tubulopathy. Bone marrow contains a 5% kappa-restricted plasma-cell clone that does not meet myeloma criteria. What treatment principle applies?

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Correct answer: CTreat the nephrotoxic clone as monoclonal gammopathy of renal significance

The best answer is “Treat the nephrotoxic clone as monoclonal gammopathy of renal significance”. The kidney lesion creates a treatment indication even without overt malignancy. Haematology and nephrology characterize the clone and choose clone-directed therapy while replacing Fanconi losses. “Observe the clone while monitoring kidney function and proteinuria” is less appropriate because MGRS is defined by kidney injury despite a small clone “Use phosphate replacement while clone-directed assessment proceeds” is less appropriate because replacement treats losses but not pathogenic light-chain production “Select clone-directed treatment from plasma-cell characterization” is less appropriate because clone-directed therapy depends on whether the clone is plasma-cell or B-cell derived “Use glucocorticoids when the renal lesion is steroid responsive” is less appropriate because nonspecific immunosuppression does not reliably suppress the responsible clone

Reference: International Kidney and Monoclonal Gammopathy Research Group consensus: https://pmc.ncbi.nlm.nih.gov/articles/PMC7136169/