• Baojian Liu, Ming Bai, Yan Wang, Di Wang, Jin Zhao, Lu Li, Ruijuan Dong, and Shiren Sun.
• Department of Nephrology, Xijing Hospital, The Fourth Military Medical University, Xi'an, Shaanxi, China.
• Eur. J. Intern. Med. 2019 Nov 1; 69: 32-41.

BackgroundThe role of bortezomib in the treatment of immunoglobulin light chain (AL) amyloidosis is not well defined. We performed this meta-analysis to evaluate the efficacy and safety of bortezomib-based regimens in patients with AL amyloidosis who are not eligible for or refuse autologous stem cell transplantation.MethodsA systematic search of Medline, Embase, and the Cochrane Library was conducted to identify related studies.ResultsTwenty-four studies with 1238 patients were included. The pooled overall response rate (ORR) and complete hematological response rate (CHR) were 0.72 (95% CI, 0.67-0.77) and 0.35 (95% CI, 0.30-0.40), respectively. Bortezomib significantly improved the outcome of ORR compared to other regimens (RR 1.28, 95% CI, 1.04-1.57, P = .02). Similar results were observed in CHR (RR 1.90, 95% CI, 1.45-2.50, P < .001) and cardiac response (RR 2.03, 95% CI, 1.31-3.13, P = .002), but not in overall survival (HR 0.82, 95% CI, 0.62-1.09, P = .17). In addition, once-weekly bortezomib was associated with improved overall survival compared with twice-weekly bortezomib (HR 0.52, 95% CI, 0.27-0.99, P = .05). Peripheral neuropathy was the most widely reported adverse event. Incorporation of bortezomib into the standard melphalan + dexamethasone setting showed a trend of increased serious adverse events, though this was not statistically significant (RR 1.29, 95% CI, 0.95-1.75, P = .10).ConclusionsCurrent evidence indicates that bortezomib-based regimens might be effective and safe therapies for patients with AL amyloidosis. There is a great need to conduct more well-designed randomized controlled trials to provide high-quality evidence.Copyright © 2019 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

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