Improvement in survival in Light chain (AL) amyloidosis has been seen over recent decades, enabling more patients to achieve long‐term survival. Patients with AL amyloidosis who survived ≥10 years from time of diagnosis (n = 186) were the subject of this study. Ten‐year survivors represented 22% of the total population. These patients were characterized by favourable patient, organ and plasma cell features. Of note, trisomies were less common among 10‐year survivors compared to those who did not survive to 10 years. All‐time best haematological response was complete response in 67%, very good partial response in 30%, partial response in 2% and no response in 1%, with 11% having received a consolidative strategy for inadequate response to first line therapy. The overall organ response rate to first‐line therapy was 76%, which increased to 86% when considering subsequent line(s) of therapy. Forty‐seven percent of the 10‐year survivors did not require a second‐line therapy. The median treatment‐free survival (TFS) among the 10‐year survivors was 10·5 years (interquartile range 7·4‐12·2). On multivariate analysis independent predictors for TFS were the achievement of complete haematological response and lack of cardiac involvement. Long‐term survivors are increasingly seen in AL amyloidosis and present distinct patient, organ and clonal disease features.

中文翻译：

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AL淀粉样变性病的十年幸存者：特征和治疗模式。

近几十年来，人们发现轻链（AL）淀粉样变性病的生存率有所提高，使更多的患者能够实现长期生存。自诊断之日起存活≥10年的AL淀粉样变性病患者（n = 186）是本研究的主题。十年幸存者占总人口的22％。这些患者的特征是患者，器官和浆细胞功能良好。值得注意的是，与没有存活到10年的人相比，在10年的幸存者中三体症的发病率较低。历来最佳的血液学反应为完全缓解率为67％，非常良好的部分缓解为30％，部分缓解为2％，无响应为1％，其中11％接受了对一线治疗反应不足的巩固策略。一线治疗的整体器官反应率为76％，考虑后续治疗时，这一比率增加至86％。10岁幸存者中有47％不需要二线治疗。10年幸存者中的无治疗生存期（TFS）为10·5年（四分位数范围7·4-12·2）。在多变量分析中，TFS的独立预测因子是完全血液学反应的实现和缺乏心脏受累。长期幸存者越来越多地出现在AL淀粉样变性病中，并呈现出明显的患者，器官和克隆性疾病特征。