Current PerspectiveSequential cyclophosphamide-bortezomib-dexamethasone unmasks the harmful cardiac effect of dexamethasone in primary light-chain cardiac amyloidosis

- Cardiac involvement in systemic light-chain amyloidosis is frequent and carries poor prognosis.
- Chemotherapy might destabilise cardiac amyloidosis during the early phase of administration.
- Dexamethasone might increase fluid retention and arrhythmia leading to cardiac events.
- Escalation-sequential administration of dexamethasone decrease cardiac-related sudden death.

Chemotherapy combining cyclophosphamide, bortezomib and dexamethasone is widely used in light-chain amyloidosis. The benefit is limited in patients with cardiac amyloidosis mainly because of adverse cardiac events. Retrospective analysis of our cohort showed that 39 patients died with 42% during the first month. A new escalation-sequential regimen was set to improve the outcomes. Nine newly-diagnosed patients were prospectively treated with close monitoring of serum N-terminal pro-brain natriuretic peptide, troponin-T and free light chains. The results show that corticoids may destabilise the heart through fluid retention. Thus, a sequential protocol may be a promising approach to treat these patients.