Prognostic staging systems for amyloidosis

Prognostic model	Risk groups		Survival in patients not undergoing stem cell transplantation		Survival in patients undergoing stem cell transplantation
Prognostic model	Risk groups		Median in months	5-year survival rate (%)*	Median in months	4-year survival rate (%)*
Mayo Stage^[1,2]	Stage I	Cardiac troponin <0.035 mcg/L and NT-proBNP <332 ng/L	26	28	Not reached at 40 months	85^¶
	Stage II	Any 1 factor high	11	12	Not reached at 40 months	75^¶
	Stage III	Cardiac troponin ≥0.035 mcg/L and NT-proBNP ≥332 ng/L	4	8	8	25^¶
Revised Mayo Stage^[3]	Stage I	NT-proBNP <1800 ng/L, cardiac troponin T <0.025 mcg/L, and difference between involved and uninvolved serum free light chains <18 mg/dL	55	50	Not reached	87
	Stage II	Any 1 factor high	19	35	97	72
	Stage III	Any 2 factors high	12	20	58	56
	Stage IV	NT-proBNP ≥1800 ng/L, cardiac troponin T ≥0.025 mcg/L, and difference between involved and uninvolved serum free light chains ≥18 mg/dL	5	15	22	46

NT-proBNP: N-terminal pro-B-type natriuretic peptide.
* Estimated from published survival curve if not reported.
¶ Survival rate at 40 months.

References:

Dispenzieri A, Gertz MA, 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.
Dispenzieri A, Gertz MA, Kyle RA, et al. Prognostication of survival using cardiac troponins and N-terminal pro-brain natriuretic peptide in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation. Blood 2004; 104:1881.
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.

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