Xu Junhui, Wang Mangju, Shen Ye, Yan Miao, Xie Weiwei, Wang Bingjie, Liu Huihui, Cen Xinan
Department of Hematology, Peking University First Hospital, Beijing, People's Republic of China.
Cancer Manag Res. 2021 Feb 11;13:1343-1356. doi: 10.2147/CMAR.S287922. eCollection 2021.
Amyloid light-chain amyloidosis (AL amyloidosis) is commonly associated with multiple myeloma. However, the clinical characteristics and prognosis of symptomatic and smoldering multiple myeloma with AL amyloidosis are not particularly clear.
Patients with symptomatic and smoldering multiple myeloma in the Peking University First Hospital registry from 2010 to 2018 were studied. The clinical and laboratory information was collected from first presentation to death or until the last available clinical follow-up. The patients' survival and outcomes were analyzed, and the relationship between the clinical parameters and survival was also assessed.
Compared with symptomatic multiple myeloma patients without AL amyloidosis, patients with AL amyloidosis had higher incidence of BNP≧700pg/mL (<0.001), ALP>187.5IU/L (=0.032) and ALB<25g/L (<0.001). Similarly, compared with smoldering multiple myeloma patients without AL amyloidosis, patients with AL amyloidosis had higher incidence of BNP≧700pg/mL (=0.030) and Alb<25g/L (=0.024). The existence of AL amyloidosis, especially those with the heart involvement, was related to shorter long-term survival of symptomatic and smoldering multiple myeloma according to univariate analyses. Renal involvement and gastrointestinal tract involvement had an impact on the prognosis of smoldering multiple myeloma but not on the symptomatic multiple myeloma. Cox regression model for overall survival detected BNP≧700pg/mL in symptomatic multiple myeloma having independent poorer prognostic significance (HR=2.455, =0.004). Interestingly, BNP at diagnosis was significantly correlated with cardiac amyloidosis (r=0.496, <0.001). Cox regression model for overall survival detected the presence of AL amyloidosis in smoldering multiple myeloma having independent poorer prognostic significance (HR=8.741, =0.002).
AL amyloidosis is an independent poor prognostic factor for not only symptomatic multiple myeloma but also smoldering multiple myeloma. It is mainly because of involvement of important organs, especially the heart. AL amyloidosis probably has a greater impact on the prognosis of smoldering multiple myeloma than on the symptomatic multiple myeloma.
淀粉样轻链淀粉样变性(AL淀粉样变性)通常与多发性骨髓瘤相关。然而,有症状和无症状多发性骨髓瘤合并AL淀粉样变性的临床特征及预后尚不完全清楚。
对2010年至2018年北京大学第一医院登记的有症状和无症状多发性骨髓瘤患者进行研究。收集从首次就诊到死亡或最后一次临床随访的临床和实验室信息。分析患者的生存情况和结局,并评估临床参数与生存之间的关系。
与无AL淀粉样变性的有症状多发性骨髓瘤患者相比,合并AL淀粉样变性的患者BNP≧700pg/mL的发生率更高(<0.001)、碱性磷酸酶>187.5IU/L(=0.032)及白蛋白<25g/L的发生率更高(<0.001)。同样,与无AL淀粉样变性的无症状多发性骨髓瘤患者相比,合并AL淀粉样变性的患者BNP≧700pg/mL的发生率更高(=0.030)及白蛋白<25g/L的发生率更高(=0.024)。单因素分析显示,AL淀粉样变性的存在,尤其是合并心脏受累,与有症状和无症状多发性骨髓瘤的长期生存时间缩短有关。肾脏受累和胃肠道受累对无症状多发性骨髓瘤的预后有影响,但对有症状多发性骨髓瘤的预后无影响。有症状多发性骨髓瘤总生存的Cox回归模型检测到BNP≧700pg/mL具有独立的较差预后意义(HR=2.455,=0.004)。有趣的是,诊断时的BNP与心脏淀粉样变性显著相关(r=0.496,<0.001)。无症状多发性骨髓瘤总生存的Cox回归模型检测到AL淀粉样变性的存在具有独立的较差预后意义(HR=8.741,=0.002)。
AL淀粉样变性不仅是有症状多发性骨髓瘤,也是无症状多发性骨髓瘤的独立不良预后因素。主要原因是重要器官受累,尤其是心脏。AL淀粉样变性对无症状多发性骨髓瘤预后的影响可能比对有症状多发性骨髓瘤的影响更大。