ArticleBlood advances2023
Predictors of treatment response and survival outcomes in patients with advanced cardiac AL amyloidosis.
Article in Blood advances, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 1 of them a synthesis that pooled it.
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Who cites it
19 citing papers in PubMed, 1 synthesis or guideline pooled it.
- The clinical trials landscape in immunoglobulin light chain amyloidosis: a systematic review.Blood advances · 2024Pooled it
- Evaluating AL-ISS within a BNP-based model for AL amyloidosis in the daratumumab era.Blood advances · 2026Article
- Tailoring heart failure management in cardiac amyloidosis.Heart failure reviews · 2026Review
- Clinical impact and prognostic implications of concurrent amyloid deposition in patients with POEMS syndrome: A single-center retrospective study.Annals of hematology · 2026Article
- Cardiac Biomarker Complete Response in AL Amyloidosis: Characteristics, Cardiac Recovery, and Survival of 63 Patients.JACC. CardioOncology · 2026Article
- [Clinical features and pathological scoring analysis of hepatic amyloidosis].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2026Article
- Economic Burden of Overall and Advanced Light Chain Amyloidosis: Results from a Claims Linked Electronic Health Record Database Analysis.ClinicoEconomics and outcomes research : CEOR · 2026Article
- On the crossroads of interdisciplinary medicine in amyloidosis - study protocol for a single-center interdisciplinary registry study.PloS one · 2026Article
- Clinical characteristics and prognosis of immunoglobulin light chain amyloidosis patients.Frontiers in medicine · 2026Article
- Kinetics of hematologic response in AL amyloidosis: Insights from clinical and cytogenetic subgroup analysis in the daratumumab era.HemaSphere · 2025Article
- Case report: recurrent chest pain as initial manifestation of rapidly progressing light-chain cardiac amyloidosis with microvascular infiltration-a novel red flag associated with poor outcomes.European heart journal. Case reports · 2025Article
- Clonal plasma cell features in light chain amyloidosis are associated with depth and timing of cardiac response independent of hematologic response.Haematologica · 2025Article
- Decoding the human tongue of light chain amyloidosis in three dimensions (3D): three-dimensional pathological visualization drives early and precise screening of suspected cases.BMC oral health · 2025Article
- Light Chain (AL) Amyloidosis Masquerading as Scleroderma: A Diagnostic Challenge.Journal of hematology · 2025Article
- Current landscape of clinical management for systemic light chain amyloidosis.Future cardiology · 2025Article
- Early dFLC response by C1D7 predicts complete hematologic response in systemic AL amyloidosis.Annals of hematology · 2025Article
- Functional Status and Quality of Life in Light-Chain Amyloidosis: Advanced Imaging, Longitudinal Changes, and Outcomes.JACC. Heart failure · 2024Article
- Early minimal residual disease eradication in light chain amyloidosis generates deeper and faster cardiac response.Clinical and experimental medicine · 2024Article
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Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Patients with advanced cardiac immunoglobulin light chain (AL) amyloidosis have a poor prognosis. Early hematologic and cardiac responses can prolong survival, but predictors of these outcomes have yet to be clarified. We report on 142 patients with newly diagnosed stage IIIb AL amyloidosis. After a median follow-up of 60 months, the median overall survival (OS) was 9 months. Independent baseline factors associated with shorter OS were symptom onset to diagnosis >6 months (hazard ratio [HR], 1.94; P = .003); bone marrow plasmacytosis ≥ 10% (HR, 1.98; P = .01); troponin I > 0.635 ng/mL (HR, 1.62; P = .04); New York Heart Association class III or IV (HR, 1.67; P = .04); and 6-minute walk test distance < 200 m (HR, 1.85; P = .01). Early hematologic (within 1 month) and cardiac (within 3 months) responses were significantly associated with longer survival. In a 1-month landmark analysis, patients with a hematologic very good partial response, partial response, and no response had a median OS of 47, 25, and 5 months, respectively (P < .0001). Patients with cardiac response at 3 months had significantly longer OS (47 vs 11 months; P < .0001). On multivariable modeling, bortezomib use was associated with early hematologic and cardiac responses and longer OS. Symptom onset to diagnosis duration of >6 months and difference between the involved and uninvolved free light chain > 350 mg/L were independently associated with lower odds of an early cardiac response. This study identified factors predictive of treatment outcomes and survival in advanced cardiac AL amyloidosis.
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