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

S. Sidana, N. Tandon, Angela Dispenzieri, Morie Gertz, F. K. Buadi, Martha Lacy, David M Dingli, A. L. Fonder, S. R. Hayman, M. A. Hobbs, Wilson Gonsalves, Y. L. Hwa, Prashant Kapoor, R. A. Kyle, N. Leung, R. S. Go, J. A. Lust, Stephen J Russell, S. R. Zeldenrust, S Vincent RajkumarShaji K Kumar

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22 Scopus citations

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 ∼ 3/45 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.

Original languageEnglish (US)
Pages (from-to)729-735
Number of pages7
JournalLeukemia
Volume32
Issue number3
DOIs
StatePublished - Mar 1 2018

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ASJC Scopus subject areas

  • Hematology
  • Cancer Research
  • Anesthesiology and Pain Medicine

Cite this

Sidana, S., Tandon, N., Dispenzieri, A., Gertz, M., Buadi, F. K., Lacy, M., Dingli, D. M., Fonder, A. L., Hayman, S. R., Hobbs, M. A., Gonsalves, W., Hwa, Y. L., Kapoor, P., Kyle, R. A., Leung, N., Go, R. S., Lust, J. A., Russell, S. J., Zeldenrust, S. R., ... Kumar, S. K. (2018). Clinical presentation and outcomes in light chain amyloidosis patients with non-evaluable serum free light chains. Leukemia, 32(3), 729-735. https://doi.org/10.1038/leu.2017.286