Case Report Effectiveness of Bortezomib in Cardiac AL Amyloidosis: A Report of Two Cases Santi Nigrelli, 1 Giuseppe Curciarello, 2 Piercarlo Ballo, 3 Stefano Michelassi, 1 and Francesco Pizzarelli 1 1 Department of Medicine, Nephrology Unit, St. M. Annunziata Hospital, ASL 10, Florence, Italy 2 Hematology and Transfusional Service, St. M. Annunziata Hospital, ASL 10, Florence, Italy 3 Cardiology Unit, St. M. Annunziata Hospital, ASL 10, Florence, Italy Correspondence should be addressed to Santi Nigrelli; nigrellisanti@hotmail.com Received 6 October 2013; Revised 3 February 2014; Accepted 5 February 2014; Published 10 March 2014 Academic Editor: Michael S. Firstenberg Copyright © 2014 Santi Nigrelli et al. his is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Cardiac involvement is a major prognostic determinant in patients with primary AL amyloidosis. he clinical results of standard therapeutic approaches are suboptimal. It has been recently shown that bortezomib, an inhibitor of the proteasome, can induce rapid favourable responses in AL amyloidosis improving cardiac function and survival. Herein we report on two patients with cardiac amyloidosis treated by bortezomib who experienced partial or total remission of hematologic disease and of cardiac involvement. However, death of one patient, sufering from chronic kidney disease stage 5, due to fulminant respiratory syndrome suggests the need for caution in bortezomib use if patients have this comorbid condition. 1. Introduction Cardiac amyloidosis, which occurs as a complication of primary amyloidosis, is a result of cardiac deposition of insol- uble, monoclonal immunoglobulin light chain fragments. his is observed in about 50% of patients with light chain amyloidosis predominantly ater 40 years of age with higher prevalence in men than in women. he development of restrictive cardiomyopathy, complicated by progressive let ventricular or biventricular dysfunction, represents a major determinant of adverse outcome for these patients who typically die as a result of progressive heart failure or sudden cardiac death. A simple staging index for cardiomyopathy, based on levels of N-terminal brain natriuretic peptide (NTproBNP) and troponin I at presentation of disease, has been shown to predict median survival of 27.2, 11.1, and 4.1 months in stages I (normal values of both markers), II (altered values of only one marker), or III (altered values of both markers), respectively [1]. he aim of cardiac amyloidosis treatment is to reduce the concentration of the amyloidogenic light chains and to induce improvement of cardiac function and survival [2, 3] and it is crucial that treatment is rapidly efective, tolerable, and safe. he severity of cardiac involvement and the high tox- icity treatment proile, however, have always limited the outcome of traditional treatment options based on high dose melphalan chemotherapy followed by autologous stem cell transplantation. Recently it has been shown that bortezomib, a proteasome inhibitor, can induce rapid responses in AL amyloidosis improving cardiac function and survival [4]. We report herein two cases of cardiac amyloidosis in which treatment with bortezomib was associated with partial or complete clinical remission of disease. 2. Case Presentation 2.1. Case Report 1. A 54-year-old man was referred to our institution in June 2006 because of clinical signs of congestive heart failure. He was a long distance runner and diver but during the last year he had experienced progressive dyspnea and fatigue in his sport activities. Hindawi Publishing Corporation Case Reports in Medicine Volume 2014, Article ID 627474, 6 pages http://dx.doi.org/10.1155/2014/627474