Abstract
It is well known that staging of patients with AL amyloidosis at diagnosis predicts for survival, but there is a paucity of literature delineating the prognostic value of these systems at relapse. We evaluated the prognostic value of AL staging among 413 patients initiated with second-line therapy between 2000 and 2015. Both the Revised Mayo 2012 and the European revision of Mayo 2004 staging systems were used. The median time from initial treatment to second-line therapy was 11.7 months. The first-line therapy was autologous stem cell transplant (ASCT) in 179 (43%) patients and non-ASCT therapies in 234 patients. Median survival from the institution of second-line therapy was 61 months. Both the Mayo 2004 and 2012 staging systems were of prognostic benefit at second-line therapy with respective risk ratios of 2.78 (95% CI: 2.15, 3.58) and 3.03 (95% CI: 2.33, 3.93) for patients with > stage 2 disease. On multivariate analysis, the predictive value of staging at second-line therapy was independent of stage at diagnosis and prior ASCT as first-line therapy. This study indicates that the Mayo staging systems work well at second-line therapy. Consequently, it is suitable for the stratification of patients in trials for relapsed, refractory AL amyloidosis.
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Acknowledgements
We thank the Predolin Foundation, the JABBS Foundation, and the support from the SPORE grant (no. CA 186781) to our work.
Author contributions
Conception and design: Yi L Hwa and Angela Dispenzieri. Provision of study materials of patients: all authors. Data analysis and interpretation: Yi L Hwa and Angela Dispenzieri. Manuscript writing: all authors. Final approval of manuscript: all authors.
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AD: received research funding from Jannsen, Takeda, Celgene, Pfizer, Alnylam, Prothena, and GSK, and serves on the advisory board of Takeda and Intellia. MAG: received funding from Amgen, Prothena, Annexon, Appellis, Johnson and Johnson, and Celgene consultancy (Milleniu), and honoraria from Celgene, Millenium, Onyx, Novartis, Smith Kline, Prothena, and Ionis. SK: received research grants for clinical trials from Celgene, Takeda, Janssen, BMS, Sanofi, KITE, Merck, Abbvie, Medimmune, Novartis, Roche-Genentech, and Amgen. DD: received research funding from Karyopharm Therapeutics, Amgen, and Millenium Pharmaceuticals. NL: serves on the advisory board of Takeda and Prothena. PK: receives funding from Takeda, Sanofi, and Amgen. MQL: received research funding from Celgene. The remaining authors declare no conflict of interest.
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Hwa, Y.L., Gertz, M.A., Kumar, S.K. et al. Prognostic restaging at the time of second-line therapy in patients with AL amyloidosis. Leukemia 33, 1268–1272 (2019). https://doi.org/10.1038/s41375-019-0400-5
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DOI: https://doi.org/10.1038/s41375-019-0400-5