AL amyloidosis: Organ response and progression criteria

Organ	Response	Progression
Heart	NT-proBNP response (>30% and >300 ng/L decrease in patients with baseline NT-proBNP ≥650 ng/L) or NYHA class response (≥2 class decrease in subjects with baseline NYHA class 3 or 4).	NT-proBNP progression (>30% and >300 ng/L increase)* or cTn progression (≥33% increase) or ejection fraction progression (≥10% decrease).
Kidney^[1]	≥30% decrease in 24-hour urine protein or drop of proteinuria below 0.5 g/24 hours in the absence of kidney progression. Creatinine and creatinine clearance must not worsen by 25% over baseline.	≥25% decrease in eGFR.
Liver	50% decrease in abnormal alkaline phosphatase value. Decrease in liver size radiographically at least 2 cm.	50% increase of alkaline phosphatase above the lowest value.
Peripheral nervous system	Improvement in electromyogram nerve conduction velocity (rare).	Progressive neuropathy by electromyography or nerve conduction velocity.

AL: light-chain; NT-proBNP: N-terminal pro-B-type natriuretic peptide; cTn: cardiac troponin; NYHA: New York Heart Association; eGFR: estimated glomerular filtration rate.

* Patients with progressively worsening renal function cannot be scored for NT-proBNP progression.

Reference:

Palladini G, Hegenbart U, Milani P, et al. A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis. Blood 2014; 124:2325.

Modified by permission from: Macmillan Publishers Ltd: Leukemia. Comenzo RL, Reece D, Palladini G, et al. Consensus guidelines for the conduct and reporting of clinical trials in systemic light-chain amyloidosis. Leukemia 2012; 26:2317. Copyright © 2012. https://www.nature.com/leu/.

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AL amyloidosis: Organ response and progression criteria