Serum N-Terminal Pro–Brain Natriuretic Peptide Is a Sensitive Marker of Myocardial Dysfunction in AL Amyloidosis

Abstract

NT-proBNP appeared to be the most sensitive index of myocardial dysfunction and the most powerful prognostic determinant in AL amyloidosis. It adds prognostic information for newly diagnosed patients and can be useful in designing therapeutic strategies and monitoring response. NT-proBNP is a sensitive marker of heart toxicity caused by amyloidogenic light chains.

Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): A consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis

“…Abnormalities in strain echocardiography [59], elevation of the N terminal Pro brain natriuretic peptide (NT-Pro BNP) [60], and elevation of cardiac troponins [61] are seen in a wide variety of cardiac disorders, and the sensitivity and specificity of these tests compared with echocardiography have not been evaluated sufficiently for these variables to be incorporated as criteria of cardiac involvement. Myocardial amyloid is excluded by normal values of NT-Pro BNP [62].…”

Consensus guidelines for the conduct and reporting of clinical trials in systemic light-chain amyloidosis

“…Patients with cardiac involvement can present with fatigue, progressive dyspnea on exertion, diastolic dysfunction, left ventricular hypertrophy in the absence of hypertension or valvular disease, pericardial effusion, and low voltage on electrocardiogram. 25 The cardiac biomarkers N-terminal prohormone of brain natriuretic peptide (NT-proBNP) 26 and troponin T (or I) 27 are prognostic with respect to survival in AL patients, and a system incorporating these biomarkers is currently in use, with patients Figure 1. Patients in this international case series from seven centers were analyzed for survival at 3 months after beginning therapy based on metrics for hematologic response assessed at 6 months.…”

Melphalan and dexamethasone with or without bortezomib in newly diagnosed AL amyloidosis: a matched case–control study on 174 patients

“…3 Clinical and experimental evidence indicate that cardiac damage in AL amyloidosis is mainly determined by a direct toxicity exerted by the circulating amyloidogenic free light chain (FLC). [4][5][6][7][8] Thus, therapy of AL amyloidosis is aimed at obtaining a rapid and profound FLC reduction by targeting the amyloidogenic plasma cell clone with chemotherapy, while attentively supporting involved organ function. Treatment approaches are derived from multiple myeloma and are adapted to the fragile amyloid patients, and novel agents are finding their place in the therapeutic armamentarium for AL amyloidosis.…”