Kyle et al listed criteria for determining if a patient with primary systemic amyloidosis have responded to therapy. The authors are from the Mayo Clinic.


Conditions evaluated:

(1) nephrotic syndrome level proteinuria (>= 3 grams protein per 24 hour)

(2) liver involvement

(3) cardiac involvement

(4) serum albumin

(5) serum monoclonal protein

(6) urine monoclonal protein excretion


Indications of Response

nephrotic syndrome level proteinuria

>= 50% reduction in proteinuria without an increase in serum creatinine (progres-sive renal insufficiency excluded)

liver involvement

decrease in extent by >= 2 cm; decrease in alkaline phosphatase by >= 50%

cardiac involvement

echocardiographic evidence of decrease in intervertebral septum >= 2 mm; increase in LVEF >= 20%

serum albumin

increase >= 1 g/dL from a pretreatment value < 3 g/dL and with stable renal function

serum monoclonal protein

disappearance or > 50% decrease

urine monoclonal protein excretion

disappearance or > 50% decrease


The decrease in liver involvement is probably in the midclavicular line.


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