Patient selection: immunoglobulin light chain amyloidosis
Parameters for evaluating light chain response:
(1) serum kappa light chains in mg/L at baseline
(2) serum lambda light chains in mg/L at baseline
(3) serum kappa light chains in mg/L after therapy
(4) serum lamda light chains in mg/L after therapy
(5) serum kappa light chains in mg/L at baseline
(6) serum lambda light chains in mg/L at baseline
(7) serum kappa light chains in mg/L after therapy
(8) serum lamda light chains in mg/L after therapy
(9) free light chain ratio after therapy
(10) immunofixation of serum
(11) immunofixation of urine
Parameters for evaluating cardiac response:
(1) NT-proBNP at baseline
(2) NT-proBNP after therapy
difference between the kappa and lambda light chain =
= ABS((kappa chain concentration) – (light chain concentration))
Parameters for evaluating cardiac function:
(1) NT-proBNP in ng/L at baseline (upper limit of normal 334 ng/L in women > 50 years of age)
(2) NT pro-BNP in ng/L after therapy
change in NT-proBNP =
= ((NT-proBNP after therapy) – (NT-proBNP at baseline)) / (NT-proBNP at baseline) * 100%
Criteria for complete light chain response:
(1) serum negative for monoclonal abnormality on immunofixation
(2) urine negative for monoclonal abnormality on immunofixation
(3) free light chain ratio normal
Criteria for very good light chain response:
(1) (difference between free light chains < 40 mg/L) AND (not complete response)
Criteria for partial response:
(1) (decrease in the difference between free light chains > 50%) AND (not very good response)
Criteria for cardiac response – all of the following:
(1) baseline NT-proBNP >= 650 ng/L
(2) > 30% decrease from baseline
(3) > 300 ng/L decrease from baseline
Criteria for cardiac progression – both of the following:
(1) > 30% increase in NT-proBNP from baseline
(2) > 300 ng/L increase
If neither criteria for cardiac status met then no change.
