Table 1.

Criteria for hematologic and organ response

Hematologic responseCriteria
Complete response (CR) Both criteria must be met:
  • Absence of amyloidogenic light chains (either free and/or as part of a complete immunoglobulin) defined by negative immunofixation electrophoresis of both serum and urine.

  • Either a FLC ratio within the reference range or the uninvolved FLC concentration is greater than involved FLC concentration with or without an abnormal FLC ratio

 
Very good partial response (VGPR) dFLC <40 mg/L 
Partial response (PR) dFLC decrease >50% 
No response (NR) Less than a partial response 
Organ response  
 Cardiac response Decrease of NT-proBNP by >30% and 300 ng/L (if baseline NT-proBNP >650 ng/L) 
 Renal response At least 30% decrease in proteinuria or drop below 0.5 g/24 h, in the absence of renal progression defined as a >25% decrease in eGFR 
 Hepatic response 50% decrease in abnormal alkaline phosphatase value or decrease in radiographic liver size by ≥2 cm 
Hematologic responseCriteria
Complete response (CR) Both criteria must be met:
  • Absence of amyloidogenic light chains (either free and/or as part of a complete immunoglobulin) defined by negative immunofixation electrophoresis of both serum and urine.

  • Either a FLC ratio within the reference range or the uninvolved FLC concentration is greater than involved FLC concentration with or without an abnormal FLC ratio

 
Very good partial response (VGPR) dFLC <40 mg/L 
Partial response (PR) dFLC decrease >50% 
No response (NR) Less than a partial response 
Organ response  
 Cardiac response Decrease of NT-proBNP by >30% and 300 ng/L (if baseline NT-proBNP >650 ng/L) 
 Renal response At least 30% decrease in proteinuria or drop below 0.5 g/24 h, in the absence of renal progression defined as a >25% decrease in eGFR 
 Hepatic response 50% decrease in abnormal alkaline phosphatase value or decrease in radiographic liver size by ≥2 cm 

Hematologic, cardiac, and renal response criteria have been validated,39,51 whereas hepatic response criteria have been obtained by consensus.52 A value adequate to measure hematologic response is deemed to be 50 mg/L. For patients with dFLC between 50 and 20 mg/L, hematologic response other than complete response (ungraded) is reached when dFLC falls below 10 mg/L.53,54

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