Divergent Clinical Phenotypes, Organ Involvement, and Survival in AL Amyloidosis and Light Chain Deposition Disease: Insights From a Large Single-Center Cohort
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This retrospective single-center cohort included 480 immunoglobulin light-chain amyloidosis (AL) and 61 light chain deposition disease (LCDD) patients from Peking University First Hospital to compare clinical profiles and outcomes. AL frequently presented with multisystem (≥ 3 organs, 31.0%) and cardiac involvement (60.8%). LCDD was kidney-dominant (eGFR < 50 mL/min/1.73m², 85.2%) with higher clonal burden (dFLC > 180mg/L, 44/60 [73.3%] with available data), a trend toward higher CR rate (65.8% vs 48.8% AL, P = 0.058), and 100% ≥ PR to daratumumab in a small subgroup (n = 11). PI-based therapy was the main backbone (66.3% vs 66.0%). LCDD had superior OS ( P < 0.001), while AL showed better renal survival in high-risk strata (albumin < 30g/L, eGFR < 50, 24h-UTP > 5g; all P < 0.05). These findings support disease-specific management: deep hematologic remission for AL and renal protection plus clone-directed therapy for LCDD.
