AIM  To investigate the clinical characteristics, therapeutic response, and risk factors that influence the prognosis of patients with light chain cardiac amyloidosis (AL-CA).

  METHODS  65 AL-CA patients who had been hospitalized in our center from September 2016 to September 2021 were retrospectively analyzed. Patients treated with bortezomib combined with dexamethasone (BD) regimen were classified as BD group (n=38), and patients treated with cyclophosphamide, thalidomide plus dexamethasone regimen or melphalan combined with dexamethasone regimen were recorded as non-BD group (n=27). Information of patients at the time of admission were collected and analyzed, including the baseline clinical data, electrocardiogram (ECG), echocardiography (EC), therapeutic response and follow-up results. COX proportional hazards model was used to identify independent prognostic factors for AL-CA patients.

  RESULTS  Compared with the non-BD group, the BD group had a higher proportion of Mayo 2012 stage IV patients and higher serum free light chain difference (P<0.01). The main manifestations of ECG in both groups were lead low voltage, the main findings of echocardiography were diastolic dysfunction and thickening of the ventricular septum or left ventricular wall, and there was no statistical difference between the groups. At 3 months, 24 patients in the BD group and 17 patients in the non-BD group had evaluable hematologic responses, with 71% and 35% of patients achieving a very good partial hematologic response or above, respectively ( P<0.05). Through COX proportional hazards model, it was found that alkaline phosphatase, D-dimer, serum creatinine, N-terminal brain natriuretic peptide precursor, serum troponin T and ejection fraction were the prognostic factors of patients. Enzyme (P<0.05, HR=1.004), serum creatinine (P<0.01, HR=1.009), and ejection fraction (P<0.05, HR=0.901) were the main factors affecting the prognosis and survival of patients.

  CONCLUSION  Low-voltage on ECG with diastolic dysfunction and thickening of ventricular septum or left ventricular wall on EC are common characteristics of AL-CA patients. First-line use of BD regimen in AL-CA patients results in a favorable hematologic response at 3 months. High alkaline phosphatase, high serum creatinine and low ejection fraction are independent risk factors for overall survival of AL-CA patients.