title: Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL.
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.
authors.affiliation: Biotechnology Research Laboratories-IRCCS Policlinico San Matteo, Piazzale Golgi 19, 27100-Pavia, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Palladini
authors.forename: Francesca
authors.initials: F
authors.lastname: Lavatelli
authors.forename: Paola
authors.initials: P
authors.lastname: Russo
authors.forename: Stefano
authors.initials: S
authors.lastname: Perlini
authors.forename: Vittorio
authors.initials: V
authors.lastname: Perfetti
authors.forename: Tiziana
authors.initials: T
authors.lastname: Bosoni
authors.forename: Laura
authors.initials: L
authors.lastname: Obici
authors.forename: Arthur R
authors.initials: AR
authors.lastname: Bradwell
authors.forename: GianVico Melzi
authors.initials: GM
authors.lastname: D'Eril
authors.forename: Roberto
authors.initials: R
authors.lastname: Fogari
authors.forename: Remigio
authors.initials: R
authors.lastname: Moratti
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000097:blood* / Q000188:drug therapy / Q000276:immunology / Q000401:mortality; D003404:Creatinine / Q000097:blood; D005260:Female; D006331:Heart Diseases / Q000209:etiology* / Q000401:mortality; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood*; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood*; D010446:Peptide Fragments / Q000097:blood*; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D007147:Immunoglobulin Light Chains; D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D003404:Creatinine