Validated staging systems for AL amyloidosis
| Staging systems . | Markers and thresholds . | Stages . | Outcomes . |
|---|---|---|---|
| Standard Mayo Clinic37 | NT-proBNP > 332 ng/L | I. No markers above the cutoff | I. Median survival 26 mo-not reached |
| cTnT > 0.035 ng/mL (or cTnI > 0.01 ng/mL) | II. One marker above the cutoff | II. Median survival 11-49 mo | |
| III. Both markers above the cutoff | III. Median survival 4-6 mo | ||
| European staging of advanced cardiac involvement38 | Standard Mayo Clinic stage III plus | a. No high-risk factors | a. Median survival 26 mo |
| Systolic blood pressure < 100 mm Hg | b. One high-risk factor | b. Median survival 6 mo | |
| NT-proBNP > 8500 ng/L | c. Two high-risk factors | c. Median survival 3 mo | |
| Revised Mayo Clinic48 | NT-proBNP > 1800 ng/L | I. No markers above the cutoff | I. Median survival 94 mo |
| cTnT > 0.025 ng/mL | II. One marker above the cutoff | II. Median survival 40 mo | |
| dFLC > 180 mg/L* | III. Two markers above the cutoff | III. Median survival 14 mo | |
| IV. Three markers above the cutoff | IV. Median survival 6 mo | ||
| Renal36 | eGFR < 50 mL/min per 1.73 m2 | I. Both eGFR above and proteinuria below the cutoffs | I. 0%-3% risk for dialysis at 2 y |
| Proteinuria > 5 g/24h | II. Either eGFR below or proteinuria above the cutoffs | II. 11%-25% risk for dialysis at 2 y | |
| III. Both eGFR below and proteinuria above the cutoffs | III. 60%-75% risk for dialysis at 2 y |
| Staging systems . | Markers and thresholds . | Stages . | Outcomes . |
|---|---|---|---|
| Standard Mayo Clinic37 | NT-proBNP > 332 ng/L | I. No markers above the cutoff | I. Median survival 26 mo-not reached |
| cTnT > 0.035 ng/mL (or cTnI > 0.01 ng/mL) | II. One marker above the cutoff | II. Median survival 11-49 mo | |
| III. Both markers above the cutoff | III. Median survival 4-6 mo | ||
| European staging of advanced cardiac involvement38 | Standard Mayo Clinic stage III plus | a. No high-risk factors | a. Median survival 26 mo |
| Systolic blood pressure < 100 mm Hg | b. One high-risk factor | b. Median survival 6 mo | |
| NT-proBNP > 8500 ng/L | c. Two high-risk factors | c. Median survival 3 mo | |
| Revised Mayo Clinic48 | NT-proBNP > 1800 ng/L | I. No markers above the cutoff | I. Median survival 94 mo |
| cTnT > 0.025 ng/mL | II. One marker above the cutoff | II. Median survival 40 mo | |
| dFLC > 180 mg/L* | III. Two markers above the cutoff | III. Median survival 14 mo | |
| IV. Three markers above the cutoff | IV. Median survival 6 mo | ||
| Renal36 | eGFR < 50 mL/min per 1.73 m2 | I. Both eGFR above and proteinuria below the cutoffs | I. 0%-3% risk for dialysis at 2 y |
| Proteinuria > 5 g/24h | II. Either eGFR below or proteinuria above the cutoffs | II. 11%-25% risk for dialysis at 2 y | |
| III. Both eGFR below and proteinuria above the cutoffs | III. 60%-75% risk for dialysis at 2 y |
cTn, cardiac troponin.
In this study, FLC were measured with the Freelite immunonephelometric assay based on polyclonal antibodies. A novel assay, based on monoclonal antibodies (N latex FLC) has been marketed in Europe and Australia. Available data indicate that the Freelite and N latex FLC assays have comparable diagnostic sensitivity and prognostic relevance. However, the 2 tests are not interchangeable, and N latex FLC results cannot be used in the staging system.