Publication

Abstract : Background: The prognosis of AL amyloidosis depends on the number and severity of organ involvement, especially cardiac. Nearly half of them die within a year of diagnosis. Chemotherapy has shown to slightly improve survival. We have compared the outcomes between the two different regimens and independent factors predicting early mortality. Methods: Retrospective analysis from 2006 - 2015 from hospital database. In patients with AL cardiac amyloidosis, cardiac involvement was defined as per American society of echocardiography (ASE) criteria. We compared the outcomes in those who received mel/pred (MP) or mel/dex (MDex) with borte, cyclo, dex (CyBorD) as induction chemotherapy and evaluated the clinical and ECHO parameters of early relapse (ER) within one year. Results: Among 50 patients of AL amyloidosis with cardiac involvement, 25 got MP/Mdex and 25 CyBorD, both matched for age and sex. 86% had >2 organs involved at diagnosis. During mean follow up of 20 months, CyBorD had higher survival rate (52% vs 32%, p = 0.023). Hematological overall response rate ORR (CR+VGPR+PR) for CYBorD was 80% with complete responses (CR) in 20%. ORR for MP/MDex was 48% with best response of VGPR in 8%. In CyBorD cohort, patients who received > 12 cycles of chemotherapy had better outcomes (70% vs 40%, p = 0.016). 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 < 0.001). In patients who completed atleast 6 months of chemotherapy, decrease in E/E1 (survivors delta E/E1 5.2+ 0.8, nonsurvivors 0.8 + 0.3, p = 0.001) was associated with better one year survival. Conclusions: Bortezomib based chemotherapy seems to improve survival and induce high rates of hematologic response. Baseline Troponin I, NYHA class III/IV, difference in FLC, restrictive pattern of diastolic dysfunction and BNP were the independent predictors of early mortality within one year in AL amyloidosis with cardiac involvement. Legal entity responsible for the study: Amrita institute of medical sciences, Kochi, India