The purpose of this study is to determine if CAEL-101 improves overall survival in patients with cardiac AL amyloidosis.

Inclusion criteria

Patients eligible for inclusion in this study must meet all of the following criteria:

• AL amyloidosis in May stage IIIA or IIIb.
• Planned first-line treatment with a cyclophosphamide-bortezomib-dexamethasone-based regimen.
• Measurable hematological disease.
• Histopathological diagnosis of amyloidosis and confirmation of AL-derived amyloid deposits
• Cardiac involvement with a diagnosis of heart failure in the context of AL amyloidosis

Exclusion criteria

• Patients eligible for this study must not meet any of the following criteria:
• Any form of amyloidosis other than AL amyloidosis, or i meet the definition of multiple myeloma or POEMS syndrome.
• Previous therapy for AL amyloidosis or multiple myeloma.
• Taking prednisone (or equivalent) >10mg/day, doxycycline or receiving dialysis.
• Supine systolic blood pressure < 90mmHg, symptomatic orthostatic hypotension.