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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. This Article Full Text Full Text (PDF) All Versions of this Article: 2005-11-4385v1 107/10/3854    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Palladini, G. Articles by Merlini, G. Search for Related Content PubMed PubMed Citation Articles by Palladini, G. Articles by Merlini, G. Related Collections Immunobiology Neoplasia Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS 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, and Giampaolo Merlini From the Center for Amyloidosis, Biotechnology Research Laboratories and Department of Biochemistry, the Department of Internal Medicine, the Clinical Chemistry Laboratory, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) San Matteo, and the University of Pavia, Pavia, Italy; the Binding Site Ltd and the Medical School, University of Birmingham, Birmingham, United Kingdom; and the Department of Medicine and Surgery, University of Milan, Milan, Italy. 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. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Monitoring organ response in amyloidosis Morie A. Gertz Blood 2006 107: 3814. [Full Text] [PDF] This article has been cited by other articles: A. Mignot, F. Bridoux, A. Thierry, S. Varnous, M. Pujo, A. Delcourt, J. M. Gombert, J.-M. Goujon, F. Favreau, G. Touchard, et al. Successful heart transplantation following melphalan plus dexamethasone therapy in systemic AL amyloidosis Haematologica, March 1, 2008; 93(3): e32 - e35. [Abstract] [Full Text] [PDF] J. B. Selvanayagam, P. N. Hawkins, B. Paul, S. G. Myerson, and S. Neubauer Evaluation and Management of the Cardiac Amyloidosis J. Am. Coll. Cardiol., November 27, 2007; 50(22): 2101 - 2110. [Abstract] [Full Text] [PDF] R. Sitia, G. Palladini, and G. Merlini Bortezomib in the treatment of AL amyloidosis: targeted therapy? Haematologica, October 1, 2007; 92(10): 1302 - 1307. [Full Text] [PDF] E. Kastritis, A. Anagnostopoulos, M. Roussou, S. Toumanidis, C. Pamboukas, M. Migkou, A. Tassidou, I. Xilouri, S. Delibasi, E. Psimenou, et al. Treatment of light chain (AL) amyloidosis with the combination of bortezomib and dexamethasone Haematologica, October 1, 2007; 92(10): 1351 - 1358. [Abstract] [Full Text] [PDF] M. A. Gertz, M. Q. Lacy, A. Dispenzieri, S. R. Hayman, S. K. Kumar, N. Leung, and D. A. Gastineau Effect of hematologic response on outcome of patients undergoing transplantation for primary amyloidosis: importance of achieving a complete response Haematologica, October 1, 2007; 92(10): 1415 - 1418. [Abstract] [Full Text] [PDF] L. M. Dember Amyloidosis-Associated Kidney Disease J. Am. Soc. Nephrol., December 1, 2006; 17(12): 3458 - 3471. [Abstract] [Full Text] [PDF] V. Sanchorawala Light-Chain (AL) Amyloidosis: Diagnosis and Treatment Clin. J. Am. Soc. Nephrol., November 1, 2006; 1(6): 1331 - 1341. [Abstract] [Full Text] [PDF] G. Merlini and M. J. Stone Dangerous small B-cell clones Blood, October 15, 2006; 108(8): 2520 - 2530. [Abstract] [Full Text] [PDF]

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