Table 5 Risk Stratification Models and Staging Systems for AL Amyloidosis.

From: Current risk stratification and staging of multiple myeloma and related clonal plasma cell disorders

Risk Stratification and Variables

Stages and proportion of patients

HR and Median OS for Mortality (95% CI)

Mayo Clinic 2004 Risk Stratification with European Modification

• Troponin T ≥ 0.035 ng/mL (1 point)

• NT-proBNP ≥332 pg/mL (1 point)

• NT-proBNP >8500 pg/mL for Stage IIIB

Stage I (Score 0): Both Troponin T and NT-proBNP below threshold

Stage II (Score 1): One of two markers above threshold

Stage III (Score 2): both markers above cutoff

European Modification (Stage IIIB): Troponin T ≥ 0.035 ng/mL and NT-proBNP>8500 pg/mL

Stage I: HR: 1.0 (reference) Median OS: 26.4 months

Stage II: HR: 2.5 Median OS: 10.5 months

Stage III: HR: 6.7 Median OS: 3.5 months

[Stage IIIA- NT-proBNP ≤8500 pg/mL: HR of 4.9]

Stage IIIB: HR: 11.1

Mayo Clinic 2012 Model

• dFLC≥18 mg/dL (1 point)

• cTnT ≥ 0.025 ng/mL (1 point)

• NT-proBNP ≥ 1800 pg/mL (1 point)

Stage I (Score 0): None of the marker above cutoff

Stage II (Score 1): One of the three markers above cutoff

Stage III (Score 2): any two markers above cutoff

Stage IV (Score 3): All three markers above cutoff

Stage I: HR:1.0 (reference) Median OS: 94.1 months [5-year OS: 59%]

Stage II: HR: 1.7 Median OS: 40.3 months [5-year OS: 42%]

Stage III: HR: 4.1 Median OS: 14 months [5-year OS: 20%]

Stage IV: HR: 6.3 Median OS: 5.8 months[5-year OS: 14%]

Renal AL staging

Stage

3-year risk of dialysis dependence

Risk Factors:

• eGFR >50 ml/min/1.73 m2 (normalized body surface area)

• 24 h proteinuria >5 g

Stage I: neither risk factor present

Stage II: one of the two risk factors present

Stage III: both risk factors present

0% in the derivation cohort

4% in the validation cohort

15% in the derivation cohort 30% in the validation cohort

60% in the derivation cohort

85% in the validation cohort

  1. cTnT cardiac troponin T, dFLC difference in involved and uninvolved serum free light chains, eGFR estimate glomerular filtration rate, HR Hazard Ratio, OS overall survival.