Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL amyloidosis
Articolo
Data di Pubblicazione:
2006
Citazione:
Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL amyloidosis / G. Palladini, F. Lavatelli, P. Russo, S. Perlini, T. Bosoni, L. Obici, A.R. Bradwell, G.V. Melzi d’Eril, R. Fogari, R. Moratti, G. Merlini. - In: BLOOD. - ISSN 0006-4971. - 107:10(2006), pp. 3854-3858.
Abstract:
N-terminal natriuretic peptide type B (NT-proBNP) is a marker of cardiac dysfunction in light chain amyloidlosis (AL) and a powerful prognostic determinant. Serum NT-proBNP and circulating free light chains (FLCs) were measured at enrollment and after 3 cycles of chemotherapy in 51 patients with cardiac AL. In patients (n = 22, 43%) in whom FLCs decreased by more than 50% (hematologic response), NT-proBNP concentration decreased by a median of 48%, whereas in the remaining patients it increased by 47% (P = .01). The reduction of NT-proBNP was greater in patients (n = 9) in whom amyloidogenic FLCs disappeared at immunofixation (median 53%), than in the remaining responding patients (median 31%, P = .04). Left ventricular wall thickness decreased by at least 2 mm in 3 of 20 patients in whom NT-proBNP improved. Fifteen patients died. Thirteen of them, in whom NT-proBNP and FLCs did not improve, died after a median of 1.8 months. The decrease of FLCs translates into a simultaneous decrease of NT-proBNP and improved survival. Patients in whom chemotherapy fails to induce such a decrease are at risk of early death. Cardiac function in AL can rapidly improve due to a reduction of the circulating amyloidogenic precursor, despite the amount of cardiac amyloid deposits remaining apparently unaltered, as measured by echocardiography.
Tipologia IRIS:
01 - Articolo su periodico
Elenco autori:
G. Palladini, F. Lavatelli, P. Russo, S. Perlini, T. Bosoni, L. Obici, A.R. Bradwell, G.V. Melzi d’Eril, R. Fogari, R. Moratti, G. Merlini
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