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Blood, 15 May 2006, Vol. 107, No. 10, pp. 3854-3858.
Prepublished online as a Blood First Edition Paper on January 24, 2006; DOI 10.1182/blood-2005-11-4385.
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Submitted November 7, 2005
Accepted January 11, 2006
Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL amyloidosis
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, and Giampaolo Merlini*
Center for Amyloidosis, Biotechnology Research Laboratories and Department of Biochemistry, Istituto di Ricovero e Cura a Carattere Scientifico San Matteo and University of Pavia, Pavia, Pavia, Italy; Department of Internal Medicine, Istituto di Ricovero e Cura a Carattere Scientifico San Matteo and University of Pavia, Pavia, Pavia, Italy
Department of Internal Medicine, Istituto di Ricovero e Cura a Carattere Scientifico San Matteo and University of Pavia, Pavia, Pavia, Italy
Clinical Chemistry Laboratory, Istituto di Ricovero e Cura a Carattere Scientifico San Matteo and University of Pavia, Pavia, Pavia, Italy
Center for Amyloidosis, Biotechnology Research Laboratories and Department of Biochemistry, Istituto di Ricovero e Cura a Carattere Scientifico San Matteo and University of Pavia, Pavia, Pavia, Italy
The Binding Site Ltd, the Medical School, The Binding Site Ltd University of Birmingham, Birmingham, United Kingdom
Department of Medicine and Surgery, University of Milan, Milan, Milan, Italy
* Corresponding author; email: gmerlini{at}unipv.it.
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 (FLC) were measured at enrollment and after three cycles of chemotherapy in 51 patients with cardiac AL. In patients (n=22, 43%) in whom FLC decreased by >50% (hematologic response), NT-proBNP concentration decreased by a median of 48%, whereas in the remaining patients it increased by 47% (p=0.01). The reduction of NT-proBNP was greater in patients (n=9) in whom amyloidogenic FLC disappeared at immunofixation (median 53%), than in the remaining responding patients (median 31%, p=0.04). Left ventricular wall thickness decreased by 2 mm in 3/20 patients in whom NT-proBNP improved. Fifteen patients died. Thirteen of them, in whom NT-proBNP and FLC did not improve, died after a median of 1.8 months. The decrease of FLC 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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