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AL Amyloidosis — RACP Adult Medicine MCQ

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ModerateHaematologyAL AmyloidosisRACP Adult Medicine

A 58-year-old man presents with nephrotic syndrome, macroglossia, and carpal tunnel syndrome. Serum free light chains show markedly elevated lambda chains. Tissue biopsy shows apple-green birefringence with Congo red staining. Bone marrow has 7% clonal plasma cells. What is the most likely diagnosis?

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Correct answer: CPrimary amyloidosis (AL)

Organ involvement (nephrotic syndrome, macroglossia, carpal tunnel) with Congo red positive amyloid deposits and clonal light chain excess is diagnostic of AL amyloidosis. Key organs affected include kidney, heart, liver, and nerves. Treatment targets the underlying plasma cell clone (typically daratumumab-VCd). Cardiac involvement determines prognosis.

Reference: IMWG – 2024 – AL Amyloidosis Consensus; BSH 2024 Amyloidosis Guidelines