A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis

Blood. 2014 Oct 9;124(15):2325-32. doi: 10.1182/blood-2014-04-570010. Epub 2014 Aug 12.


The kidney is involved in 70% of patients with immunoglobulin light-chain (AL) amyloidosis, but little is known on progression or reversibility of renal involvement, and criteria for renal response have never been validated. Newly diagnosed patients from the Pavia (n = 461, testing cohort) and Heidelberg (n = 271, validation cohort) centers were included. Proteinuria >5 g/24 h and estimated glomerular filtration rate (eGFR) <50 mL/min predicted progression to dialysis best. Proteinuria below and eGFR above the thresholds indicated low risk (0 and 4% at 3 years in the testing and validation cohorts, respectively). High proteinuria and low eGFR indicated high risk (60% and 85% at 3 years). At 6 months, a ≥25% eGFR decrease predicted poor renal survival in both cohorts and was adopted as criterion for renal progression. A decrease in proteinuria by ≥30% or below 0.5 g/24 h without renal progression was the criterion for renal response, being associated with longer renal survival in the testing and validation populations. Hematologic very good partial or complete remission at 6 months improved renal outcome in both populations. We identified and validated a staging system for renal involvement and criteria for early assessment of renal response and progression in AL amyloidosis that should be used in clinical practice and trial design.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Amyloidosis / drug therapy*
  • Amyloidosis / pathology*
  • Biomarkers / metabolism*
  • Cohort Studies
  • Dialysis
  • Disease Progression
  • Female
  • Humans
  • Immunoglobulin Light Chains / metabolism*
  • Immunoglobulin Light-chain Amyloidosis
  • Kidney / pathology*
  • Male
  • Middle Aged
  • Proportional Hazards Models
  • Reproducibility of Results
  • Risk Factors
  • Survival Analysis
  • Time Factors
  • Treatment Outcome


  • Biomarkers
  • Immunoglobulin Light Chains