Biomarker models in use in systemic amyloidosis
| . | Risk factor thresholds . | Stages* . | Hazard ratio† for death (95%CI) . | ||
|---|---|---|---|---|---|
| Model . | cTnT, μg/L . | NT-proBNP, ng/L . | Other factors . | ||
| AL amyloidosis | |||||
| Mayo 2004 model | ≥0.035‡ | ≥332 | None | 1 | Reference |
| 2 | 2.5 (1.9-3.5) | ||||
| 3 | 6.7 (5.0-9.1) | ||||
| European 2015 modification of Mayo 2004 model | ≥0.035‡ | ≥332 | Stage 3 only: NT-proBNP >8500 ng/L | 1 | Reference |
| 2 | 2.6 (1.9-3.5) | ||||
| 3a | 4.9 (3.6-6.8) | ||||
| 3b | 11.1 (8.1-15.4) | ||||
| Mayo 2012 model | ≥0.025 | ≥1800§ | Difference between involved to uninvolved light chain ≥180 mg/L | 1 | Reference |
| 2 | 1.7 (1.2-2.3) | ||||
| 3 | 4.1 (3.1-5.5) | ||||
| 4 | 6.3 (4.8-8.3) | ||||
| ATTR amyloidosis | |||||
| Mayo model | >0.05 | >3000 | None | 1 | Reference |
| 2 | 1.4 (0.8-2.6) | ||||
| 3 | 3.6 (2.0-6.4) | ||||
| NAC model | — | >3000 | eGFR <45 mL/min/1.73 m2 | 1 | Reference |
| 2 | 2.1 (1.5-2.8) | ||||
| 3 | 3.8 (2.7-5.3) | ||||
| . | Risk factor thresholds . | Stages* . | Hazard ratio† for death (95%CI) . | ||
|---|---|---|---|---|---|
| Model . | cTnT, μg/L . | NT-proBNP, ng/L . | Other factors . | ||
| AL amyloidosis | |||||
| Mayo 2004 model | ≥0.035‡ | ≥332 | None | 1 | Reference |
| 2 | 2.5 (1.9-3.5) | ||||
| 3 | 6.7 (5.0-9.1) | ||||
| European 2015 modification of Mayo 2004 model | ≥0.035‡ | ≥332 | Stage 3 only: NT-proBNP >8500 ng/L | 1 | Reference |
| 2 | 2.6 (1.9-3.5) | ||||
| 3a | 4.9 (3.6-6.8) | ||||
| 3b | 11.1 (8.1-15.4) | ||||
| Mayo 2012 model | ≥0.025 | ≥1800§ | Difference between involved to uninvolved light chain ≥180 mg/L | 1 | Reference |
| 2 | 1.7 (1.2-2.3) | ||||
| 3 | 4.1 (3.1-5.5) | ||||
| 4 | 6.3 (4.8-8.3) | ||||
| ATTR amyloidosis | |||||
| Mayo model | >0.05 | >3000 | None | 1 | Reference |
| 2 | 1.4 (0.8-2.6) | ||||
| 3 | 3.6 (2.0-6.4) | ||||
| NAC model | — | >3000 | eGFR <45 mL/min/1.73 m2 | 1 | Reference |
| 2 | 2.1 (1.5-2.8) | ||||
| 3 | 3.8 (2.7-5.3) | ||||
For each stage, stage I is absence of any risk factors; stage II is presence of 1 risk factor; stage III is presence of 2 risk factors, and where applicable, stage IV is all risk factors present. The exception is the European 2015 modification of the Mayo 2004 model in which stage III patients (2 risk factors) are further divided by whether NT-proBNP is >8500 ng/L.
Hazards for the AL amyloidosis models derived from a recent data set (E.M., Terry M. Therneau, Dirk Larson, M.A.G., Martha Q. Lacy, Francis Buadi, David Dingli, Suzanne R. Hayman, Prashant Kapoor, Wilson I. Gonsalves, Taxiarchis V. Kourelis, Rahma Warsame, Amie Fonder, Miriam Hobbs, Yi Lisa Hwa, Nelson Leung, Stephen J. Russell, John A. Lust, Yi Lin, Ronald S. Go, Steven R. Zeldenrust, Robert A. Kyle, S. Vincent Rajkumar, S.K.K., and A.D., manuscript submitted September 2018). Hazard ratios for the ATTR Mayo model and NAC model are derived from the original publications.5,7
Alternatively, cTnI ≥100 ng/L (Dade-Behring, Newark, DE).
Alternatively, BNP ≥400 ng/L.