Staging of immunoglobulin light chain (AL) amyloidosis

Model	Risk factor thresholds	Stage		Hazard ratio for death (95% CI)*
Mayo 2004^[1]	Troponin: Cardiac TnT ≥0.035 mcg/L or Cardiac TnI ≥0.1 mcg/L or High sensitivity cardiac TnT ≥50 ng/L BNP: NT-proBNP ≥332 ng/L or BNP ≥81 ng/L	Stage I	No risk factors	Reference
		Stage II	1 risk factor	2.5 (1.9-3.5)
		Stage III	2 risk factors	6.7 (5.0-9.1)
Mayo 2004 with European modification^[2]	Troponin: Cardiac TnT ≥0.035 mcg/L or Cardiac TnI ≥0.1 mcg/L or High sensitivity cardiac TnT ≥50 ng/L BNP: NT-proBNP ≥332 ng/L or BNP ≥81 ng/L	Stage I	No risk factors	Reference
		Stage II	1 risk factor	2.5 (1.9-3.5)
		Stage IIIA	2 risk factors NT-proBNP 332 to <8500 ng/L or BNP 81 to <700 ng/L	4.9 (3.6-6.8)
		Stage IIIB	2 risk factors NT-proBNP ≥8500 ng/L or BNP ≥700 ng/L	11.1 (8.1-15.4)
Mayo 2012^[3]	Troponin Cardiac troponin T ≥0.025 mcg/L or High sensitivity cardiac troponin T ≥40 ng/L BNP: NT-proBNP ≥1800 ng/L or BNP ≥400 ng/L dFLC ≥18 mg/dL	Stage I	No risk factors	Reference
		Stage II	1 risk factor	1.7 (1.2-2.3)
		Stage III	2 risk factors	4.1 (3.1-5.5)
		Stage IV	3 risk factors	6.3 (4.8-8.3)
Boston University^[4]	Troponin: Cardiac troponin I >0.10 ng/mL BNP: BNP >81 pg/mL	Stage I	No risk factors	Median OS >12 years
		Stage II	1 risk factor	Median OS 9.4 years
		Stage IIIA	2 risk factors BNP >81 pg/mL	Median OS 4.3 years
		Stage IIIB	2 risk factors BNP >700 pg/mL	Median OS 1 year

TnT: troponin T; TnI: troponin I; BNP: brain natriuretic peptide; NT-proBNP: N-terminal pro-brain natriuretic peptide; dFLC: difference between involved and uninvolved serum free light chains; OS: overall survival.
* Hazard ratios presented reflect use of cardiac troponin T and NT-proBNP.

References:

Dispenzieri A, Gertz M, Kyle RA, et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol 2004; 22:3751.
Palladini G, Sachchithanantham S, Milani P, et al. A European collaborative study of cyclophosphamide, bortezomib, and dexamethasone in upfront treatment of systemic AL amyloidosis. Blood 2015; 126:612.
Kumar S, Dispenzieri A, Lacy MQ, et al. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol 2012; 30:989.
Lilleness B, Ruberg FL, Mussinelli R, et al. Development and validation of a survival staging system incorporating BNP in patients with light chain amyloidosis. Blood 2019; 133:215.

Adapted from: Muchtar E, Kumar SK, Gertz M, et al. Staging systems use for risk stratification of systemic amyloidosis in the era of high-sensitivity troponin T assay. Blood 2019; 133:763.

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Staging of immunoglobulin light chain (AL) amyloidosis