A comprehensive overview of AL amyloidosis disease characteristics accumulated over two decades at a single referral center in Korea

Background Characteristics of AL amyloidosis across Asia are not well-described in the literature. Thus, we overviewed the incidence and disease characteristics of AL amyloidosis in Korea. Methods We collected medical records of 302 AL amyloidosis patients and compared survival outcomes by predominant treatment strategy and at four time points: 1995–2003, 2004–2008, 2009–2013, and 2014–2018. Results The median age was 62 years (36–83). One hundred forty-one patients were classified as stage III (26.3%) or IV (47.9%). The patients diagnosed between 2014 and 2018 survived longer than those diagnosed at other time points due to the introduction of bortezomib ( p < 0.01). In addition, patients who received upfront ASCT survived longer than those who received salvage ASCT or chemotherapy alone ( p < 0.01). However, most of the 85 patients who experienced early death within 6 months were older than 75 years, had BMI less than 20, and had a high disease burden. Conclusions The incidence of AL amyloid has increased and survival outcomes have improved gradually, most likely due to introduction of novel agents and upfront ASCT. However, not all patients are suitable for these potent treatment modalities, and avoiding early death within 6 months remains a challenge.