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

Research output: Contribution to journalArticlepeer-review

34 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

ASJC Scopus subject areas

  • Hematology
  • Cancer Research
  • Anesthesiology and Pain Medicine

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