• Yoshitaka Kikukawa, Hiromichi Yuki, Sinya Hirata, Kazuhiko Ide, Hirotomo Nakata, Toshikazu Miyakawa, Naofumi Matsuno, Kisato Nosaka, Yuji Yonemura, Tatsuya Kawaguchi, Hiroyuki Hata, Hiroaki Mitsuya, and Yutaka Okuno.
• Department of Hematology, Rheumatology & Infectious Diseases, Kumamoto University of Medicine, 1-1-1 Honjo, Kumamoto, 860-8556, Japan.
• Int. J. Hematol. 2015 Feb 1; 101 (2): 133-9.

AbstractAmyloid light-chain amyloidosis (ALA) is a rare disease with poor prognosis and is often associated with monoclonal gammopathy of undetermined significance, multiple myeloma, or Waldenström macroglobulinemia. Only high-dose melphalan with auto-peripheral blood stem cell transplantation (PBSCT) has shown high long-term hematological response rates, but combinations with novel agents, including bortezomib or lenalidomide, have recently shown high hematological response rates for AL amyloidosis patients. In the present study, we treated eight Japanese patients with AL amyloidosis using bortezomib, cyclophosphamide, and dexamethasone (CyBorD). Overall response rate was 100 %; four patients (50 %) had complete remissions (CR), two (25 %) had very good partial responses, and two (25 %) had partial responses. Five of six patients (83 %) had organ responses in the heart and/or kidney. A relapsed patient repeatedly achieved CR with the CyBorD treatment. One patient died of sudden cardiac arrest a month after normalization of his serum free light chain level, which may be attributable to his spending the previous 6 months undergoing PBSCT collection and high-dose melphalan with auto-PBSCT. Altogether, the CyBorD regimen achieved high levels of hematological responses relatively quickly (within 2-3 months). The CyBorD regimen, rather than high-dose melphalan treatment, could serve as a first-line therapy for Japanese patients with ALA.

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