AL Amyloidosis Treatment — ESENeph MCQ

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HardChronic Kidney DiseaseAL Amyloidosis TreatmentESENeph

A 60-year-old woman presents with nephrotic syndrome and biopsy shows amyloid deposits (Congo red positive). Mass spectrometry typing reveals AL amyloid (lambda light chain). Serum free light chains show markedly elevated lambda. What is the first-line treatment?

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Correct answer: A — Clone-directed bortezomib-based therapy

AL amyloidosis requires treatment of the underlying clonal plasma cell disorder. Bortezomib-based regimens (typically CyBorD: Cyclophosphamide, Bortezomib, Dexamethasone) are first-line for AL amyloidosis. The goal is deep haematological response (normalisation of free light chain ratio). Colchicine is used for AA amyloidosis (FMF). Organ transplantation alone does not address the underlying disease. Anti-CD38 antibodies (Daratumumab) are increasingly used based on ANDROMEDA trial results. The competing options do not fit the renal phenotype, temporal relationship, treatment threshold or safety constraint described. Management still requires confirmation of current medicines, renal trajectory and relevant contraindications, followed by timely biochemical and clinical reassessment. Escalate urgently if life-threatening electrolyte disturbance, respiratory compromise, shock, neurological deterioration or a dialysis indication develops.

Reference: BSH 2020 – AL Amyloidosis; NICE 2022 – Daratumumab for AL Amyloidosis: https://kdigo.org/wp-content/uploads/2024/03/KDIGO-2024-CKD-Guideline.pdf