Table 2.
Current Prognostic Staging Scores for ATTR and AL CA
| ATTR | AL | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| NAC | Mayo* | Columbia | BU | Mayo | ||||||
| Staging/scoring parameters | NT-proBNP > 3000 pg/mL and eGFR < 45 mL/min/1.73 m2 | NT-proBNP > 3000 pg/mL and Troponin T > 0.05 ng/mL | NYHA class I–IV (1 to 4 points), Daily dose of furosemide or equivalent: > 1 mg/kg (3 points), >0.5–1 mg/kg (2 points), > 0–0.5 mg/kg (1 point), and 0 mg/kg (0 points), or NAC or Mayo score (0–2 points) | BNP > 81 pg/mL and Troponin I > 0.1 ng/mL | NT-proBNP ≥ 1800 pg/mL, Troponin T ≥ 0.025 ng/mL, FLC-diff ≥ 18 mg/dL | |||||
| Stage | Median survival | Stage | 4-year survival/median survival | Score | Median survival | Stage | Median survival | Stage | 5-year survival | |
| Stage III (2 parameters) | 24.1 months | Stage III (2 parameters) | 57% / 66 months | Score 7–9 | 19.8 months (NAC), 0.3 months (Mayo) | Stage IIIb (BNP > 700 pg/mL with 2 parameters) | 12 months | Stage IV (3 parameters) | 14% | |
| Stage II (1 parameter) | 46.7 months | Stage II (1 parameter) | 42% / 40 months | Score 4–6 | 36 months (NAC), 38.5 months (Mayo) | Stage III (2 parameters) | 51.6 months | Stage III (2 parameters) | 28% | |
| Stage I (0 parameters) | 69.2 months | Stage I (0 parameters) | 18% / 20 months | Score 1–3 | 90.5 months | Stage II (1 parameter) | 112.8 months | Stage II (1 parameter) | 60% | |
| – | – | – | – | – | – | Stage I (0 parameters) | Not reached | Stage I (0 parameters) | 68% | |
| Reference | [110] | [111] | [112] | [75] | [113] | |||||
Note: *For ATTRwt and not ATTRv.
Abbreviations: BU, Boston University School of Medicine; NYHA, New York Heart Association; NT-proBNP, N-terminal pro-B-type natriuretic peptide; NAC, UK National Amyloidosis Center; FLC-diff, difference between involved and uninvolved free light chain; eGFR, estimated glomerular filtration rate; ATTR, transthyretin amyloidosis; AL, light-chain amyloidosis.