机构地区:[1]大连医科大学附属第一医院检验科 [2]大连市中心医院检验科,辽宁大连116000
出 处:《中国实验血液学杂志》2025年第2期593-600,共8页Journal of Experimental Hematology
摘 要:目的:回顾性分析轻链型淀粉样变患者的临床特征、预后情况及预后影响因素,为该病的诊治提供参考依据。方法:收集2017年1月至2022年11月在2家医院确诊为轻链型淀粉样变的52例患者的临床数据。分析患者的临床特征、死亡组与存活组患者的临床指标差异,采用Kaplan-Meier曲线进行生存分析,采用Cox回归模型分析影响预后的因素。结果:52例患者的中位年龄为61(41-81)岁,男性患者占63.5%。心脏(69.2%)和肾脏(67.3%)是最易受累器官,67.3%的患者有两个以上器官受累。大多数患者(71.2%)使用含有硼替佐米的化疗方案,其中包括5例(9.6%)接受了达雷妥尤单抗联合硼替佐米治疗的患者。死亡组的男性患者比例(81.0%)、心脏受累比例(95.2%)、梅奥2012分期≥Ⅲ期比例(95.2%)、hs-cTnI水平及NT-proBNP水平均明显高于存活组患者(P<0.05)。入组患者的中位生存时间为33.4(2.6-60.2)个月,1、2、3和5年生存率分别为83.7%、79.3%、58.9%及32.7%。Kaplan-Meier生存曲线分析显示,男性(P=0.040)、NT-proBNP≥3600 ng/L(P<0.001)、梅奥2012分期≥Ⅲ期(P<0.001)及心脏受累(P=0.008)的患者预后差,生存时间短。多因素回归分析结果显示,梅奥2012分期≥Ⅲ期是预后的独立危险因素。结论:近年来轻链型淀粉样变患者的生存率明显提高,但5年以上生存率仍然较低。反映心脏受累程度的生物标志物NT-proBNP和hs-cTnI及梅奥2012分期仍然是患者生存有意义的预测指标。以硼替佐米为基础的化疗是目前最常用的治疗方案,添加达雷妥尤单抗的治疗方案在逐年增多。Objective:To retrospectively analyze the clinical characteristics,prognosis and prognostic factors of patients with light-chain(AL)amyloidosis,so as to provide reference for the diagnosis and treatment of AL amyloidosis.Methods:Clinical data of 52 patients diagnosed with AL amyloidosis at two hospitals from January 2017 to November 2022 were collected.The clinical characteristics,differences in clinical indexes between the deceased group and the survival group were analyzed.Kaplan-Meier curves were used for overall survival(OS)analysis,and Cox regression models were used to analyze the factors affecting the prognosis.Results:The median age of the 52 patients at diagnosis was 61(41-81)years old,and 63.5%of the patients were male.Heart(69.2%)and kidney(67.3%)were the most involved organs,and 67.3%of the patients had two or more organs involved.Most patients(71.2%)received chemotherapy regimens containing bortezomib,including 5 patients(9.6%)who received treatment with daratumumab in combination with bortezomib.The proportion of male patients(81.0%),the proportion of patients with cardiac involvement(95.2%),and the proportion of patients with Mayo 2012 stage≥III(95.2%),as well as the levels of hs-cTnI and NT-proBNP in the deceased group were significantly higher than those in the survival group(P<0.05).The median OS time of the enrolled patients was 33.4(2.6-60.2)months,with 1-year,2-year,3-year and 5-year OS rates of 83.7%,79.3%,58.9%and 32.7%,respectively.The Kaplan-Meier survival curve analysis revealed that patients with male gender(P=0.040),NT-proBNP≥3600 ng/L(P<0.001),Mayo 2012 stage≥III(P<0.001),and cardiac involvement(P=0.008)had poor prognosis and shorter overall survival(OS)time.The multivariate regression analysis showed that Mayo 2012 stage≥III was an independent risk factor for prognosis.Conclusion:In recent years,the survival rate of patients with AL amyloidosis has improved significantly,but the 5-year survival rate is still relatively low.Cardiac biomarkers(NT-proBNP and hs-cTnI)and Mayo 2012
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