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Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL.

Giovanni Palladini·Francesca Lavatelli·Paola Russo·Stefano Perlini·Vittorio Perfetti·Tiziana Bosoni·Laura Obici·Arthur R Bradwell·GianVico Melzi D'Eril·Roberto Fogari·Remigio Moratti·Giampaolo Merlini
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Abstract

N-terminal natriuretic peptide type B (NT-proBNP) is a marker of cardiac dysfunction in light chain amyloidosis (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.

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AdultAgedAged, 80 and overAmyloidosisCreatinineFemaleHeart DiseasesHeart VentriclesHumansImmunoglobulin Light ChainsMaleMiddle AgedNatriuretic Peptide, BrainPeptide FragmentsPrognosisRetrospective StudiesSurvival AnalysisTreatment OutcomeJournal ArticleResearch Support, Non-U.S. Gov't

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Giovanni Palladini, Francesca Lavatelli, Paola Russo, Stefano Perlini, Vittorio Perfetti, Tiziana Bosoni, Laura Obici, Arthur R Bradwell, GianVico Melzi D'Eril, Roberto Fogari, Remigio Moratti, Giampaolo Merlini. 2006-01-24. Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL.. https://doi.org/10.1182/blood-2005-11-4385

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