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目的:评估沙利度胺联合地塞米松化疗方案对初次治疗的系统性轻链型(AL)淀粉样变性患者的疗效和安全性。方法:回顾性分析2009年4月至2014年12月在南京军区南京总医院肾脏科确诊并采用沙利度胺联合地塞米松(TD)作为初次治疗方案的AL型淀粉样变性患者的临床及随访资料。应用Kaplan-Meier法计算患者生存时间,COX风险比例模型分析预后危险因素。结果:本研究共纳入患者63例,男女比例为1.52∶1,中位年龄为57岁,中位治疗周期为19月(4~78月)。38例患者(60.3%)心脏受累,38例(60.3%)患者两个或以上的器官受累。37例患者取得血液学反应,总体血液学缓解率为58.7%,中位缓解时间为4月,其中18例(28.6%)达到完全缓解,中位缓解时间为3月,10例(15.9%)达到非常好的部分缓解,中位缓解时间为5.5月,9例(14.3%)达到部分缓解,中位缓解时间为5月。23例(36.5%)患者获得器官缓解,肾脏缓解和心脏缓解分别为23例(36.5%)和10例(15.9%),中位缓解时间分别为8.5月和18.8月。有3例患者病情复发,中位复发时间为7月(3~19月)。中位随访时间为28月,2年生存率为78.5%,K-M曲线估计的中位生存时间尚未达到。达到血液学缓解的患者组相比未达到血液学缓解的患者组预后更好(3年生存率87.5%vs 36.3%,P<0.01)心脏受累的患者组相比于无心脏受累患者组预后更差(3年生存率52.4%vs 87.0%,P<0.01)。常见不良反应包括外周神经病变(23.8%)、胃肠道反应(17.4%)、乏力(11.1%)、皮疹(6.3%)等。9例患者因不良反应和治疗无效停药,12例患者沙利度胺减量,22例患者地塞米松减量。结论:TD方案对初次治疗的AL淀粉样变性患者有一定的疗效,且耐受性较好,长期疗效有待进一步观察。
OBJECTIVE: To evaluate the efficacy and safety of thalidomide plus dexamethasone chemotherapy in first-time patients with systemic light chain (AL) amyloidosis. Methods: The clinical data and clinical data of patients with AL amyloidosis diagnosed in the Department of Nephrology, Nanjing General Hospital of Nanjing Military Region from April 2009 to December 2014 were retrospectively analyzed. The clinical data and clinical data of patients with AL amyloidosis treated with thalidomide combined with dexamethasone (TD) Follow-up information. Kaplan-Meier method was used to calculate the survival time of patients, COX risk proportional model analysis of prognostic risk factors. Results: A total of 63 patients were enrolled in this study. The male / female ratio was 1.52: 1, the median age was 57 years and the median treatment period was 19 months (4 to 78 months). 38 patients (60.3%) had heart involvement and 38 (60.3%) patients had two or more organs involved. Thirty-seven patients achieved hematologic response. The overall hematological response rate was 58.7%. The median time to remission was 4 months. Eighteen patients (28.6%) achieved complete remission. The median time to remission was 3 months and 10 patients (15.9% Reached a very good partial response, the median response time was 5.5 months, 9 patients (14.3%) achieved partial response, the median response time was May. Twenty-three patients (36.5%) had organ remission, renal remission and cardiac remission were 23 (36.5%) and 10 (15.9%), respectively. The median time to remission was 8.5 months and 18.8 months respectively. Three patients had a relapse and the median time to relapse was July (March-September). The median follow-up time was 28 months and the 2-year survival rate was 78.5%. The median survival time estimated by the K-M curve was not yet reached. Patients who achieved hematologic remission had a better prognosis than those who did not achieve hematologic response (3-year survival 87.5% vs 36.3%, P <0.01), compared with patients without cardiac involvement Poor (3-year survival rate 52.4% vs 87.0%, P <0.01). Common adverse reactions include peripheral neuropathy (23.8%), gastrointestinal reaction (17.4%), fatigue (11.1%), rash (6.3%) and so on. Nine patients discontinued treatment because of adverse reactions and treatment, 12 patients thalidomide reduction, 22 patients dexamethasone reduction. Conclusion: The TD regimen has certain curative effect on patients with AL amyloidosis who are treated for the first time, and the tolerability is better. The long-term therapeutic effect needs to be further observed.