Clinical Features and Outcome of Primary Amyloidosis in Korea Kihyun Kim, Seok Jin Kim, Hyun Jung Jun,Yeung-Chul Mun, Chul Soo Kim, Jong-Ho Won, Soo-Mee Bang, Jae Hoon Lee

Primary Amyloidosis a monoclonal plasma cell disorder the accumulation of monoclonal light chain fragments that have undergone a conformational transformation and deposit as amyloid fibrils in different tissues. Organ dysfunction such as heart, kidney, liver, peripheral nervous system are common clinical features Primary Amyloidosis in Korea –No data about epidemiology or clinical characteristics or treatment outcome are available.

Objectives and Methods To describe clinical features and outcome of primary amyloidosis in Korea Diagosed from 1995 to 2010 Multicenter : 6 centers Retrospective chart review

Patient characteristics

Treatment

Overall Survival N=88 Median 21.6 months

P=0.003 Median OS 24.8 vs vs. 3.3 months Age 65 P=0.67 Year of diagnosis

Hematologic Response P=0.001 Median OS NR vs months Organ Response P=0.009 Median OS NR vs months Cardiac Involvement P<0.001 Median OS 38.2 vs. 8.4 months SC Transplantation P=0.03 Median OS NR vs months

Renal Involvement P=0.268 Hepatic Involvement P=0.003 Median OS 23.9 vs. 3.3 months No of Organ Involvement P=0.002 Median OS 43.8 vs vs. 9.2 months BM Plasma cell 30% P=0.049 Median OS 22.6 vs. 8.5 months

iFLC high vs. low P=0.139 NYHA FC 1 vs 2 or more P=0.003 Median OS 29.0 vs. 4.5 months NT-proBNP normal vs. abnormal P=0.023 ECOG PS 0, 1 vs 2 or more P<0.001 Median OS 24.8 vs. 3.3 months

Prognostic Factors

Summary & Conclusion We could described clinical features and outcome of Korean amyloidosis patients. The most common treatment regimen were akylating agent plus dexamethasone or predinisolone. Hematologic response, organ response, cardiac involvement, hepatic involvement, number of organ involvement, extent of BM plamacytosis, stem cell transplantation, NYHA functional grade, ECOG performance status, NT-proBNP were significant for overall survival in univariate analysis, and Hematologic response and ECOG performance status were significant prognostic in multivariate analysis.

Acknowledgement Kihyun Kim, Seok Jin Kim, Hyun Jung Jun –Samsung Medical Center, Sungkyunkwan University School of Medicine Yeung-Chul Mun –Ewha Womans University School of Medicine Chul Soo Kim –Comprehensive Cancer Center, Inha University Hospital Jong-Ho Won –Soonchunhyang University Hospital Soo-Mee Bang –Seoul National University Bundang Hospital Jae Hoon Lee –Gachon University Gil Hospital