Staging systems for AL amyloidosis
| Staging system . | Markers and thresholds . | Stages . | Outcomes* . |
|---|---|---|---|
| Cardiac (NT-proBNP based) | NT-proBNP >332 ng/L cTnT >0.035 ng/mL (or cTnI >0.01 ng/mL) | I. No markers above the cutoff | I. Median survival not reached, 57% with 10-y survival |
| II. One marker above the cutoff | II. Median survival 67 mo | ||
| IIIa. Both markers above the cutoff and NT-proBNP <8500 ng/L | IIIa. Median survival 15 mo | ||
| IIIb. Both markers above the cutoff and NT-proBNP ≥8500 ng/L | IIIb. Median survival 4 mo | ||
| Cardiac (BNP based) | BNP >81 ng/L cTnI >0.1 ng/mL | I. No markers above the cutoff | I. Median survival 151 mo, 57% with 10-y survival |
| II. One marker above the cutoff | II. Median survival 53 mo | ||
| IIIa. Both markers above the cutoff and BNP <700 ng/L | III. Median survival 13 mo | ||
| IIIb. Both markers above the cutoff and BNP ≥700 ng/L | IV. Median survival 4 mo | ||
| Revised Mayo Clinic | NT-proBNP >1800 ng/L cTnT >0.025 ng/mL dFLC >180 mg/L | I. 0 markers above the cutoff | I. Median survival not reached, 57% with 10-y survival |
| II. 1 marker above the cutoff | II. Median survival 69 mo | ||
| III. 2 markers above the cutoff | III. Median survival 16 mo | ||
| IV. 3 markers above the cutoff | IV. Median survival 6 mo | ||
| Renal | eGFR <50 mL/min per 1.73 m2 | I. Both eGFR above and proteinuria below the cutoffs | I. 1% risk of dialysis at 2 y |
| proteinuria >5 g per 24 h | II. Either eGFR below or proteinuria above the cutoffs | II. 12% risk of dialysis at 2 y | |
| III. Both eGFR below and proteinuria above the cutoffs | III. 48% risk of dialysis at 2 y |
| Staging system . | Markers and thresholds . | Stages . | Outcomes* . |
|---|---|---|---|
| Cardiac (NT-proBNP based) | NT-proBNP >332 ng/L cTnT >0.035 ng/mL (or cTnI >0.01 ng/mL) | I. No markers above the cutoff | I. Median survival not reached, 57% with 10-y survival |
| II. One marker above the cutoff | II. Median survival 67 mo | ||
| IIIa. Both markers above the cutoff and NT-proBNP <8500 ng/L | IIIa. Median survival 15 mo | ||
| IIIb. Both markers above the cutoff and NT-proBNP ≥8500 ng/L | IIIb. Median survival 4 mo | ||
| Cardiac (BNP based) | BNP >81 ng/L cTnI >0.1 ng/mL | I. No markers above the cutoff | I. Median survival 151 mo, 57% with 10-y survival |
| II. One marker above the cutoff | II. Median survival 53 mo | ||
| IIIa. Both markers above the cutoff and BNP <700 ng/L | III. Median survival 13 mo | ||
| IIIb. Both markers above the cutoff and BNP ≥700 ng/L | IV. Median survival 4 mo | ||
| Revised Mayo Clinic | NT-proBNP >1800 ng/L cTnT >0.025 ng/mL dFLC >180 mg/L | I. 0 markers above the cutoff | I. Median survival not reached, 57% with 10-y survival |
| II. 1 marker above the cutoff | II. Median survival 69 mo | ||
| III. 2 markers above the cutoff | III. Median survival 16 mo | ||
| IV. 3 markers above the cutoff | IV. Median survival 6 mo | ||
| Renal | eGFR <50 mL/min per 1.73 m2 | I. Both eGFR above and proteinuria below the cutoffs | I. 1% risk of dialysis at 2 y |
| proteinuria >5 g per 24 h | II. Either eGFR below or proteinuria above the cutoffs | II. 12% risk of dialysis at 2 y | |
| III. Both eGFR below and proteinuria above the cutoffs | III. 48% risk of dialysis at 2 y |
dFLC, difference between involved (amyloidogenic) and uninvolved circulating free light chain.
Observed in 1378 patients with AL amyloidosis newly diagnosed at the Pavia Amyloidosis Research and Treatment Center from 2004 through 2018.