Table 2.

Suggestions for clinical trial design in AL amyloidosis

Key considerations in trial design: 
  • Uniform implementation of measurable disease criteria in trials of relapsed/refractory AL amyloidosis

 
  • Preference for time-limited therapy to reduce physical and financial toxicity

 
  • Response-driven de-escalation strategies

 
  • Inclusion of patients with advanced disease (eg, NT-proBNP >8500 pg/mL, ESRD, and autonomic neuropathy)

 
  • Incorporate correlative studies on novel assays for measuring tumor burden (eg, free light chain-mass spectrometry)

 
  • Incorporate patient-reported outcome assessment to measure HR-QoL

 
  • Functional assessment (6MWT) with clinically meaningful differences

 
Key areas of unmet need: 
  • Standardization of supportive care regimens

 
  • Rare entities such as immunoglobulin M amyloidosis

 
  • Treatment and natural history studies of localized AL amyloidosis

 
  • Determine the most appropriate imaging modalities for assessing disease burden and response, considering factors such as sensitivity, specificity, and feasibility

 
  • Therapies targeting amyloid fibrils and misfolded light chains

 
Key considerations in trial design: 
  • Uniform implementation of measurable disease criteria in trials of relapsed/refractory AL amyloidosis

 
  • Preference for time-limited therapy to reduce physical and financial toxicity

 
  • Response-driven de-escalation strategies

 
  • Inclusion of patients with advanced disease (eg, NT-proBNP >8500 pg/mL, ESRD, and autonomic neuropathy)

 
  • Incorporate correlative studies on novel assays for measuring tumor burden (eg, free light chain-mass spectrometry)

 
  • Incorporate patient-reported outcome assessment to measure HR-QoL

 
  • Functional assessment (6MWT) with clinically meaningful differences

 
Key areas of unmet need: 
  • Standardization of supportive care regimens

 
  • Rare entities such as immunoglobulin M amyloidosis

 
  • Treatment and natural history studies of localized AL amyloidosis

 
  • Determine the most appropriate imaging modalities for assessing disease burden and response, considering factors such as sensitivity, specificity, and feasibility

 
  • Therapies targeting amyloid fibrils and misfolded light chains

 

6MWT, 6-minute walk test; ESRD, end-stage renal disease.

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