Immunoglobulin light chain amyloidosis (AL amyloidosis) is a monoclonal plasma cell proliferative disorder that is characterized by tissue deposits of misfolded insoluble κ and λ light chain derived amyloid fibrils, leading to organ dysfunction. The prognosis of patients depends on the number and severity of organ involvement, especially cardiac involvement. Nearly half of the patients with amyloidosis die within a year of diagnosis. We analysed factors predicting early mortality (within one year) in patients with systemic immunoglobulin light chain cardiac amyloidosis.
Retrospective analysis of patients between January 2007 and January 2016 from our hospital database. In patients with AL cardiac amyloidosis, cardiac involvement was defined as per American society of echocardiography (ASE) criteria. We evaluated the clinical, ECG and ECHO parameters of early relapse (ER) within one year in these cardiac AL amyloidosis patients. Log rank test was done to identify independent predictors of one year all cause mortality.
Among the 72 patients (mean age 58.2 + 8.2 years, 59.7% males) of AL amyloidosis with cardiac involvement, 32 (44.4%) died within one year. Sixty five patients (90%) received melphalan/thalidomide/bortezomib/cyclophosphamide/dexamethasone based chemotherapy as a monotherapy or in combinations. Logistic regression analysis revealed NYHA Class III/IV (p = 0.024), BNP (p = 0.031), Troponin I (p = 0.042), Free light chain difference (p = 0.011) and restrictive pattern of diastolic dysfunction (E/E1 > 20) to be independent predictors of all cause mortality (p = 0.021). Kaplan Meir survival analysis showed a worse prognosis in patients with E/E1 > 20 (Log rank, p
AL Cardiac amyloidosis carries a poor outcome. Baseline Troponin I, NYHA class III/IV, difference in free light chain, restrictive pattern of diastolic dysfunction and BNP were the independent predictors of early mortality within 1 year in patients with AL cardiac amyloidosis. In patients who completed atleast 6 months of chemotherapy, a decrease in the ECHO parameter E/E1 is associated with better one year survival. This will help us in better prognostication of patients with AL cardiac amyloidosis.
Clinical trial identification
This is not a clinical trial
Legal entity responsible for the study
Amrita institute of medical sciences
All authors have declared no conflicts of interest.