沙利度胺联合地塞米松治疗系统性轻链淀粉样变性的疗效
发布时间：2019-06-14 22:40
【摘要】：目的:系统性轻链型淀粉样变性(systemic immunoglobulin light chain amyloidosis,AL)是临床上最常见,也是预后最差的一种淀粉样变性。化疗和自体干细胞移植是该病的主要治疗手段,国外小样本研究表明沙利度胺联合地塞米松(thalidomide combined dexamethasone,TD)化疗方案对复发和难治性AL患者有一定的疗效,但国内外尚缺乏TD方案对初治AL患者的疗效和毒副反应的报道。本研究旨在通过回顾性分析AL患者的临床和预后资料,探讨TD方案对初治AL的疗效和安全性。方法:回顾性分析2009年4月至2014年12月在南京总医院确诊并采用TD方案作为初治方案的63例AL型淀粉样变性患者的临床及随访资料。所有患者均采用TD方案治疗4个疗程及以上,沙利度胺起始治疗剂量为50mg/晚,1周后未见不良反应则加量至100mg/晚,以此类推,最高剂量为200mg/晚,地塞米松起始治疗剂量为40mg/周,28天为1个治疗周期。应用Kaplan-Meier法计算患者生存时间,组间对比采用log-rank检验,利用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个(4-78个)。38例患者(60.3%)心脏受累,38例患者(60.3%)两个或以上的器官受累。37例患者取得血液学缓解,总体血液学缓解率为58.7%,中位到达缓解的时间为4个月,其中18例患者(28.5%)达到完全缓解(complete response,CR),中位到达缓解的时间为3个月,10例患者(15.9%)达到非常好的部分缓解(very good partial response,VGPR),中位到达缓解的时间为5.5个月,9例患者(14.2%)达到部分缓解(partial response,PR),中位到达缓解的时间为5个月。23例患者(36.5%)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%),中位到达缓解的时间分别为8.5和18.8个月。有3例患者病情复发,中位复发时间为7个月(3-19个月)。患者的中位随访时间为28个月,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相比未达到血液学缓解的患者预后更好(3年生存率87.5%vs.36.3%,P0.01),心脏受累的患者相比无心脏受累的患者预后更差(3年生存率52.4%vs.87%,P0.05)。常见不良反应包括外周神经病变(23.8%)、胃肠道反应(17.5%)、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方案对初治的AL型淀粉样变性患者有一定的疗效,并具有较好的耐受性,可以作为不适合行大剂量马法兰联合自体外周血干细胞移植(high-dose melphalan/autologous peripheral blood stem cell transplantation,HDM/ASCT)及硼替佐米联合地塞米松(bortezomib combined dexamethasone,BD)方案化疗患者的备选方案,其长期疗效有待进一步观察。
[Abstract]:Objective: systemic light chain amyloidosis (systemic immunoglobulin light chain amyloidosis,AL) is the most common and poor prognosis in clinic. Chemotherapy and autologous stem cell transplantation are the main treatments for the disease. Small sample studies abroad have shown that thalidomide combined with dexamethasone (thalidomide combined dexamethasone,TD) chemotherapy regimen has certain efficacy in patients with recurrent and refractory AL, but there is still a lack of reports on the efficacy and side effects of TD regimen in patients with newly diagnosed AL. The purpose of this study was to analyze the clinical and prognostic data of AL patients and to explore the efficacy and safety of TD regimen in the treatment of newly diagnosed AL. Methods: from April 2009 to December 2014, the clinical and follow-up data of 63 patients with AL type amyloidosis diagnosed in Nanjing General Hospital and treated with TD regimen were analyzed retrospectively. the clinical data of 63 patients with AL type amyloidosis diagnosed in Nanjing General Hospital from April 2009 to December 2014 were analyzed retrospectively. All patients were treated with TD regimen for 4 courses or more. The initial dose of thalidomide was late for 50mg/, but no adverse reaction was found until the late 100mg/ after 1 week, and so on, the highest dose was 200mg/ late, the initial dose of dexamethasone was 40mg/ week, and 28 days was a treatment cycle. Kaplan-Meier method was used to calculate the survival time of the patients, log-rank test was used to compare the groups, and COX risk ratio model was used to analyze the prognostic risk factors. Results: a total of 63 patients were enrolled in this study, the male to female ratio was 1.52 鈮，
本文编号：2499753
[Abstract]:Objective: systemic light chain amyloidosis (systemic immunoglobulin light chain amyloidosis,AL) is the most common and poor prognosis in clinic. Chemotherapy and autologous stem cell transplantation are the main treatments for the disease. Small sample studies abroad have shown that thalidomide combined with dexamethasone (thalidomide combined dexamethasone,TD) chemotherapy regimen has certain efficacy in patients with recurrent and refractory AL, but there is still a lack of reports on the efficacy and side effects of TD regimen in patients with newly diagnosed AL. The purpose of this study was to analyze the clinical and prognostic data of AL patients and to explore the efficacy and safety of TD regimen in the treatment of newly diagnosed AL. Methods: from April 2009 to December 2014, the clinical and follow-up data of 63 patients with AL type amyloidosis diagnosed in Nanjing General Hospital and treated with TD regimen were analyzed retrospectively. the clinical data of 63 patients with AL type amyloidosis diagnosed in Nanjing General Hospital from April 2009 to December 2014 were analyzed retrospectively. All patients were treated with TD regimen for 4 courses or more. The initial dose of thalidomide was late for 50mg/, but no adverse reaction was found until the late 100mg/ after 1 week, and so on, the highest dose was 200mg/ late, the initial dose of dexamethasone was 40mg/ week, and 28 days was a treatment cycle. Kaplan-Meier method was used to calculate the survival time of the patients, log-rank test was used to compare the groups, and COX risk ratio model was used to analyze the prognostic risk factors. Results: a total of 63 patients were enrolled in this study, the male to female ratio was 1.52 鈮，
本文编号：2499753
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