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AL Amyloidosis — RACP Adult Medicine MCQ

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ModerateHaematologyAL AmyloidosisRACP Adult Medicine

A 65-year-old man presents with nephrotic syndrome, restrictive cardiomyopathy (increased wall thickness, low voltages on ECG), macroglossia, and periorbital purpura. Serum free light chains show markedly elevated lambda. Bone marrow shows 8% clonal plasma cells. Fat pad aspirate shows Congo red-positive amyloid with apple-green birefringence under polarised light. What is the most likely amyloid type?

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Correct answer: C — AL amyloidosis

Nephrotic syndrome, restrictive cardiomyopathy, macroglossia, periorbital purpura, and monoclonal lambda light chains with Congo red-positive amyloid is AL (immunoglobulin light chain) amyloidosis. Macroglossia and periorbital purpura are highly specific for AL type. Treatment targets the plasma cell clone: daratumumab-bortezomib-cyclophosphamide-dexamethasone (Dara-VCd) per ANDROMEDA trial.

Reference: BSH – 2024 – Amyloidosis; ANDROMEDA Trial