He Donghua, Guan Fangshu, Hu Minli, Zheng Gaofeng, He Jingsong, Han Xiaoyan, Yang Yang, Hong Pan, Wang Gang, Zhao Yi, Wu Wenjun, Cai Zhen
Department of Hematology, Bone Marrow Transplantation Center, The First Affiliated Hospital, School of Medicine, Zhejiang University, No. 79, Qingchun Rd., Hangzhou, 310003 Zhejiang Province China.
Department of Hematology, Taizhou Hospital of Zhejiang Province, Taizhou, China.
Indian J Hematol Blood Transfus. 2022 Jul;38(3):444-453. doi: 10.1007/s12288-021-01469-y. Epub 2021 Jul 28.
To retrospectively identify the critical characteristics and prognostic factors of light-chain amyloidosis.
Data were collected and compared from 91 patients who were diagnosed with light-chain amyloidosis at four hospitals between January 2010 and November 2018. We analyzed the clinical characteristics and performed an overall survival (OS) analysis.
Patients (median age, 60 years) were diagnosed with organ involvement of the kidney (91.2%), heart (56%), liver (14.3%), soft tissue (18.7%), or gastrointestinal tract (15.4%), and 68.1% of patients had more than two organs involved. Patients were most treated with bortezomib-based regimens (56%), and only one patient had autologous stem cell transplantation (auto-ASCT). The median OS was 36.33 months and was influenced by the ECOG score, renal involvement, cardiac involvement, hepatic involvement, and persistence of positive immunofixation. Patients who received bortezomib-based treatment had a trend of favorable OS compared to those who received non-bortezomib-based treatments, but the difference was not statistically significant. Although the overall number of organs involved was not related to OS, the number of organs involved in the heart, liver and kidney was related. Multivariate analysis indicated that cardiac involvement and negative hematologic response with persistence of positive immunofixation were independent prognostic factors for OS.
Cardiac involvement and the hematologic response to treatment were independent prognostic factors for OS in light-chain amyloidosis patients.
回顾性确定轻链淀粉样变性的关键特征和预后因素。
收集并比较了2010年1月至2018年11月期间在四家医院被诊断为轻链淀粉样变性的91例患者的数据。我们分析了临床特征并进行了总生存期（OS）分析。
患者（中位年龄60岁）被诊断为肾脏（91.2%）、心脏（56%）、肝脏（14.3%）、软组织（18.7%）或胃肠道（15.4%）器官受累，68.1%的患者有两个以上器官受累。患者大多接受基于硼替佐米的方案治疗（56%），仅有1例患者进行了自体干细胞移植（auto-ASCT）。中位OS为36.33个月，受东部肿瘤协作组（ECOG）评分、肾脏受累、心脏受累、肝脏受累和免疫固定阳性持续存在的影响。与接受非硼替佐米治疗的患者相比，接受硼替佐米治疗的患者有OS良好的趋势，但差异无统计学意义。虽然受累器官总数与OS无关，但心脏、肝脏和肾脏受累器官数量与OS有关。多因素分析表明，心脏受累和免疫固定阳性持续存在的血液学阴性反应是OS的独立预后因素。
心脏受累和治疗的血液学反应是轻链淀粉样变性患者OS的独立预后因素。
