多发性骨髓瘤合并轻链型淀粉样变性患者的临床特征及预后分析

Clinical characteristics and prognosis of multiple myeloma patients associated light chain amyloidosis

目的:分析多发性骨髓瘤合并轻链型(AL型)淀粉样变性患者的临床特征、治疗和预后。方法:回顾性分析2009年7月至2022年12月在东部战区总医院国家肾脏疾病临床医学研究中心确诊的多发性骨髓瘤合并AL型淀粉样变性患者的临床资料、治疗反应及预后特征。结果:71例患者中主要以水肿(84.5%)起病,以IgG型M蛋白最常见,中位浆细胞比例15%。70例(98.6%)患者累及肾脏,41例(57.7%)累及心脏。本组患者主要接受含硼替佐米(32.4%)或沙利度胺(25.4%)的方案治疗,可评估患者总体血液学缓解率为75.0%,其中完全缓解率为8.3%,非常好的部分缓解率为38.9%,部分缓解率为27.8%;仅1例(6.7%)患者取得心脏缓解,15例(41.7%)患者取得肾脏缓解。中位随访时间为16(1.0~120.0)月,中位生存时间为34月,6月、1年、2年和4年的累积生存率分别为83.6%、75.2%、62.2%和43.0%。年龄、浆细胞比例和氨基末端脑钠肽前体水平与患者预后独立相关。结论:多发性骨髓瘤合并AL型淀粉样变性患者总体预后不佳。抗浆细胞治疗有效,但器官缓解率较低,年龄、心脏受累严重程度和肿瘤负荷是影响患者预后的独立危险因素。

Objective:To analyze the clinical features,treatment and prognosis of multiple myeloma patients associated with systemic light chain(AL)amyloidosis.Methodology:Multiple myeloma patients associated with AL amyloidosis confirmed by biopsy at Jinling Hospital from July 2009 to December 2022 were studied.We retrospectively analyzed the clinical features,treatment and prognosis of the patients.Results:Among the 71 patients,the primary presenting symptom was edema(84.5%),with IgG type M protein being the most common.The median plasma cell proportion was 15%.The majority of patients received treatment regimens containing bortezomib(32.4%)or thalidomide(25.4%).The overall hematologic response rate in the evaluated patients was 75.0%,including a complete response rate of 8.3%,a very good partial response rate of 38.9%,and a partial response rate of 27.8%.One(1/15,6.7%)patient achieved cardiac response and 15(15/36,41.7%)patients achieved renal response.The median follow⁃up time was 16(1.0~120.0)months,and the median survival time was 34 months.The survival rates at 6 months,1 year,2 years,and 4 years were 83.6%,75.2%,62.2%,and 43.0%,respectively.Age,plasma cell ratio,and N⁃terminal pro⁃brain natriuretic peptide levels were independently associated with patient prognosis.Conclusion:Patients with multiple myeloma associated with AL amyloidosis have a dismal overall prognosis.Anti⁃plasma cell therapy is effective but organ response rates are low.Age,severity of cardiac involvement,and tumor burden are independent risk factors for patient outcome.