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AL Amyloidosis Emergency Recognition — EECC MCQ

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ModerateCardiomyopathyAL Amyloidosis Emergency RecognitionEECC

A 55-year-old woman with unexplained biventricular HF undergoes investigation. CMR shows widespread subendocardial LGE with elevated T1 and ECV >55%. Echocardiography shows 'granular sparkling' myocardium with LVH. Her serum free light chains are abnormal (kappa:lambda ratio 0.05). What type of amyloidosis is this?

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Correct answer: B — AL (light chain) amyloidosis — the abnormal kappa:lambda ratio indicates clonal light chain production; this is a haematological emergency requiring urgent referral because untreated AL cardiac amyloidosis has a median survival of ~6 months

AL amyloidosis is caused by clonal plasma cell proliferation producing misfolded immunoglobulin light chains. The abnormal free light chain ratio (kappa:lambda 0.05, normal 0.26-1.65 — in this case suppressed kappa suggesting excess lambda free light chains) is diagnostic. AL cardiac amyloidosis is a MEDICAL EMERGENCY: (1) median survival ~6 months without treatment for cardiac-stage III (NT-proBNP >332 pg/mL + troponin elevation); (2) treatment: chemotherapy targeting the clonal plasma cells (bortezomib-based regimens, daratumumab + VCd, autologous stem cell transplant in selected patients); (3) cardiac support: diuretics carefully (avoid over-diuresis — preload-dependent fixed stroke volume); avoid beta-blockers and vasodilators; (4) CRITICAL: ATTR must be distinguished from AL because treatment is completely different (tafamidis for ATTR vs chemotherapy for AL). DPD scintigraphy + monoclonal protein studies enables non-invasive differentiation (Grade 2-3 DPD WITH negative monoclonal protein = ATTR).

Reference: ESC (2023): Cardiomyopathies Guidelines