Light-chain amyloidosis (AL) with cardiac involvement carries a poor prognosis; median untreated survival is<6 months. Most of AL are treated with chemotherapy associating an alkylating agent, corticoid and proteasome inhibitor.

Our study evaluates the effect on mortality of first-line chemotherapy administered in combined or in sequential in patients with cardiac AL.

Two hundred and sixty-two patients diagnosed with AL amyloidosis were retrospectively studied, since 2008. Among these patients, 34% already received chemotherapy and 15% were not treated or treated in another hospital. 51% patients newly diagnosed had a medical follow-up in our center, of whom 3% died before receiving chemotherapy. A total of 131 patients were analyzed. Initial chemotherapies administered were classified into 4 groups according to the sequence of administration: combination of Velcade (Bortzezomib)/Cyclophosphamide/Dexamethasone (VADcomb), sequential administration of Dexamethasone/Velcade/Cyclophosphamide (DCVseq) or Cyclophosphamide/Velcade/Dexamethasone (CVDseq) and other chemotherapy. Survival was assessed using a log-rank test (Fig. 1, Table 1).

Among 131 treated patients (65,5years±11.6, 63% male, 73% lambda subtype), 57 received CVDseq, 18 DCVseq, 38 VADcomb and 18 other chemotherapy. At 1 month, mortality was 16.5%, and mortality per group was 17% in CVDseq group, 16.7% in DCVseq group, 16.7% in other chemotherapy group and 15.8% in VADcomb group. Overall mortality is 45% and mortality per group is 40% with CVDseq, 44% with DCV seq, 50% with VADcomb and 50% with other chemotherapy.

Overall mortality and mortality at 1 month were not significantly different between the 4 groups (respectively P=0.785 and P=0.99). There is no difference of mortality in the cohort whether the chemotherapy is administered sequentially or in combination.