Clinical features and treatment outcome of primary systemic light-chain amyloidosis in Korea: Results of multicenter analysis

Primary systemic light-chain (AL) amyloidosis a disorder characterized by accumulation of monoclonal light chains as aggregated amyloid fibrils in tissues of multiple organs to cause organ dysfunction and death (Kyle and Gertz, Semin Hematol 1995;32:45-59; Merlini and Bellotti, N Engl J Med 2003;349:583-596). Although there are quite a number of data regarding clinical features and treatment outcomes of AL amyloidosis, most of them are from western countries except for a couple of reports from Japan (Kyle and Gertz, Semin Hematol 1995;32:45-59; Yamazaki et al., Clin Exp Nephrol 2009;13:522-525; Goodman et al., Br J Haematol 2006;134:417-425; Michael et al., Clin Lymphoma Myeloma Leuk 2010;10:56-61; Palladini et al., Blood 2004;103:2936-2938). Considering the effect of ethnic difference on the clinical course and outcomes of AL amyloidosis, the analysis of Asian patients may help better understanding of this disease entity. Therefore, we conducted retrospective analysis of clinical features and treatment outcomes of 84 newly diagnosed AL amyloidosis patients in six referral centers in Korea between 1995 and 2010.