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Multiple myeloma, gammopathies

Clinical presentation and outcomes in light chain amyloidosis patients with non-evaluable serum free light chains

Leukemia volume 32, pages 729–735 (2018)Cite this article

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Abstract

Hematologic response criteria in light chain (AL) amyloidosis require the difference in involved and uninvolved free light chains (dFLC) to be at least 5 mg/dl. We describe the clinical presentation and outcomes of newly diagnosed amyloidosis patients with dFLC <5 mg/dl (non-evaluable dFLC; 14%, n=165) compared with patients with dFLC ⩾5 mg/dl (evaluable dFLC; 86%, n=975). Patients with non-evaluable dFLC had less cardiac involvement (40% vs 80%, P<0.001), less liver involvement (11% vs 17%, P=0.04) and a trend toward less gastrointestinal involvement (18% vs 25%, P=0.08). However, significantly higher renal involvement (72% vs 56%, P=0.0002) was observed in the non-evaluable dFLC cohort. Differences in treatment patterns were observed, with 51% of treated patients undergoing upfront stem cell transplantation in the non-evaluable cohort compared with 28% in the evaluable dFLC group (P<0.001). Progression-free survival (61 vs 13 months, P<0.001) and overall survival (OS; 101 vs 29 months, P<0.001) were significantly longer in the non-evaluable dFLC cohort. Normalization of involved light chain levels and decrease in dFLC <1 mg/dl (baseline at least 2 mg/dl) were predictive of OS and associated with better dialysis-free survival and may be used for response assessment in patients with non-evaluable FLC levels.

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Acknowledgements

Author contributions

SS and SKK designed the study, collected the data, analyzed the data, wrote the first draft and approved the final version of the manuscript; NT collected the data, critically reviewed the first draft and approved the final version of the manuscript; AD, MAG, MQL, FKB, DD, ALF, MAH, SRH, WIG, YLH, PK, RAK, NL, RSG, JAL, SJR, SRZ and SVR critically reviewed the first draft and approved the final version of the manuscript.

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  1. Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA

    S Sidana, N Tandon, A Dispenzieri, M A Gertz, F K Buadi, M Q Lacy, D Dingli, A L Fonder, S R Hayman, M A Hobbs, W I Gonsalves, Y L Hwa, P Kapoor, R A Kyle, N Leung, R S Go, J A Lust, S J Russell, S R Zeldenrust, S V Rajkumar & S K Kumar

  2. Division of Nephrology, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA

    N Leung

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Correspondence to S K Kumar.

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Sidana, S., Tandon, N., Dispenzieri, A. et al. Clinical presentation and outcomes in light chain amyloidosis patients with non-evaluable serum free light chains. Leukemia 32, 729–735 (2018). https://doi.org/10.1038/leu.2017.286

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