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原发性浆细胞白血病患者临床特点及药物和造血干细胞移植的疗效

The clinical characteristics of patients with primary plasma cell leukemia and the efficacy of novel agents and hematopoietic stem cell transplantation

摘要:

目的:分析原发性浆细胞白血病(pPCL)免疫分型、细胞遗传学特点,评估硼替佐米及造血干细胞移植(HSCT)疗效及对患者生存的影响。方法:对北京大学人民医院1998年1月至2019年3月收治的42例pPCL患者临床资料进行回顾性队列研究。所有患者随访至2019年12月31日。免疫分型及细胞遗传学特点与既往已报道多中心多发性骨髓瘤免疫分型及细胞遗传学特点进行比较,应用卡方检验进行分析。将39例接受治疗患者分为硼替佐米为基础化疗组(29例)及非硼替佐米为基础化疗组(10例),应用卡方检验进行疗效比较,Kaplan-Meier方法进行单因素预后分析,Cox比例风险模型进行多因素分析。将39例患者分为造血干细胞移植组(15例)及未行造血干细胞移植组(24例),应用Kaplan-Meier方法进行单因素预后分析,Cox比例风险模型进行多因素分析。结果:42例pPCL占同期收治浆细胞疾病总体2.6%。男22例、女20例,中位发病年龄50(30~77)岁。免疫分型显示pPCL患者表达CD38、CD138、CD45、CXCR4常见。其CXCR4表达比例高于多发性骨髓瘤(73.1%比34.7%, P =0.000),CD9、CD200 表达比例低于多发性骨髓瘤(40.7%比62.5%, P=0.028,33.3%比58.0%, P =0.021)。细胞遗传学分析示50%(14/28)的患者出现核型异常。疗效分析示硼替佐米为基础的化疗方案疗效优于非硼替佐米为基础的化疗(69.0%比50.0%)。中位生存期18.2(0.2~95.7)个月,1年及2年生存率分别为61.9%、37.4%。多因素预后分析示对预后影响有统计学意义的因素为年龄( P =0.027)及疗效( P =0.035)。造血干细胞移植患者生存优于单纯化疗患者(中位生存期26.8比8.1个月, P=0.021)。 结论:pPCL患者细胞遗传学异常高于多发性骨髓瘤患者。硼替佐米的应用可提高缓解率,延长生存,HSCT的应用可能存在生存获益。

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abstracts:

Objective:To analyze the immunophenotype and cytogenetic characteristics of primary plasma cell leukemia (pPCL), and to evaluate the efficacy of bortezomib and hematopoietic stem cell transplantation as main treatment.Methods:A retrospective cohort study was conducted including 42 pPCL patients admitted to Peking University People′s Hospital from January 1998 to March 2019. All patients were followed up until December 31, 2019. The immunophenotype and cytogenetic characteristics were compared with historical data of multiple myeloma (MM). Thirty-nine patients were divided into bortezomib-based group (29 cases) and non-bortezomib group (10 cases). All patients were also divided into hematopoietic stem cell transplantation (HSCT) group (15 cases) and non-HSCT group (24 cases).Chi-square test was used for efficacy comparison, and Kaplan-Meier method was used for univariate prognostic analysis. Cox proportional hazards model was used for multi-variant analysis.Results:pPCL accounted for 2.6% of the total patients with plasma cell diseases during the same period. There were 22 males and 20 females, with a median age of 50 (30—77) years old at diagnosis. In immunophenotype analysis, tumor cells in pPCL patients also expressed CD38, CD138, CD45, which was similar as patients with MM. However the expression of CXCR4 were more frequently seen in pPCL(73.1% vs. 34.7%, P= 0.000), while intensity of CD9 and CD200 was lower (40.7% vs. 62.5%, P =0.028, 33.3% vs. 58.0%, P=0.021).Overall response rate of bortezomib-based therapy was superior to non-bortezomib therapy (69.0% vs.50.0%). The median survival was 18.2 (0.2—95.7)months, and the 1-and 2-year survival rates were 61.9% and 37.4%, respectively. Multivariate prognostic analysis suggested that age ( P= 0.027) and efficacy( P= 0.035)were significantly correlated with survival.HSCT resulted in superior survival compared with chemotherapy alone(26.8 vs. 8.1 months, P=0.021). Conclusions:Immunophenotypes and cytogenetic abnormalities in patients with pPCL are different from those with multiple myeloma. Bortezomib based regimens improve response rate and survival of pPCL. Hematopoietic stem cell transplantation also predicts survival benefits.

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期刊: 《中华内科杂志》2020年59卷10期 801-806页 MEDLINEISTICPKUCSCDCA
栏目名称: 论著
DOI: 10.3760/cma.j.cn112138-20200306-00201
发布时间: 2020-09-28
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