Prompt Treatment by a Multidisciplinary Team of Experts Contributes to the Health of “Longer Term” Survivors of Light Chain Amyloidosis

For patients with light chain cardiac amyloidosis (AL), there is a median untreated survival rate of 6 months, with heart failure serving as a primary contributor to a bleak prognosis.

At the Mayo Clinic, a prognostic staging system has been established that incorporates troponin T, N-terminal pro B-type natriuretic peptide (NT-proBNP), and the difference in serum free light chains. For patients with the most advanced Mayo stages, median survival is 14 months for stage III and about 5.8 months for stage IV. Developments in the therapeutic armamentarium including anti–plasma cell therapies have positively impacted treatment outcomes.

In this study, Andrej Gabrovsek, MD, and colleagues at the Cleveland Clinic investigated factors that contributed to extended survival in some patients with AL. A survival of ≥3 years was arbitrarily set as “longer-term,” and the researchers sought to determine the proportion of patients who achieved this. The team identified patients with AL who were seen at the clinic during a 19-year span (1997-2016). By using extra-cardiac biopsy plus diagnostic cardiac imaging or endomyocardial biopsy and/or biomarkers, a diagnosis of AL was determined.

In these patients, a Mayo stage was calculated based on requisite data when available, and survival was determined over 5 years based on each stage.

In total, 316 patients with AL were included in the study, and a total of 144 had adequate information to calculate Mayo stage.

For each stage over 5 years, number and survival information was determined and included in a table.

The notable group of survivors of ≥3 years included 81 (26%) patients. In this group, 57% were male, 69% had multiorgan involvement, and at diagnosis the average age was 61 ± 11 years. Forty-one patients had Mayo staging data available; 11 were stage IV, 17 were stage III, 9 were stage II, and 4 were stage I.

A small group of 16 patients experienced an exceptionally remarkable long-term survival of ≥8 years (5% of the overall cohort of 316). Based on these data and their clinical experience, the authors concluded that in patients with AL who present with more advanced disease, a select group of patients can achieve long-term survival of ≥3 years. This reinforces that the prompt treatment of AL by an experienced multidisciplinary clinical team is critical to improve survival outcomes.


Gabrovsek A, Donnelly J, Anwer F, et al. Longer-term survivors of light chain cardiac amyloidosis. Presented at: 2020 International Symposium on Amyloidosis; September 14-18, 2020. Abstract PT120.

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