浆细胞白血病的遗传学异常及预后因素分析  

Prognosis and Genetic Characteristics of Patients with Plasma Cell Leukemia

作　　者：武永莉 王楠[1] 鄢颖朝 袁丽君[1] 王莉莉[1] 李红华[1] 李猛[1] WU Yong-Li;WANG Nan;YAN Ying-Zhao;YUAN Li-Jun;WANG Li-Li;LI Hong-Hua;LI Meng(Department of Hematology,The First Medical Center,Chinese PLA General Hospital,Beijing 100853,China)

机构地区：[1]解放军总医院第一医学中心血液科,北京100853

出　　处：《中国实验血液学杂志》2020年第6期1804-1810,共7页Journal of Experimental Hematology

基　　金：解放军总医院临床科研扶持基金项目(2016FC-CXYY-1007)。

摘　　要：目的:分析浆细胞白血病(PCL)患者的临床特征、遗传学异常、治疗效果及相关预后因素,以提高对该病的认识。方法:回顾性分析1993年1月至2019年12月于本院确诊为浆细胞白血病的30例患者的临床表现、实验室检查、治疗方案及生存资料。结果:30例PCL患者中位年龄56.5(28-80)岁,25例为原发性浆细胞白血病,5例为继发性浆细胞白血病。细胞遗传学异常以复杂核型和亚二倍体为主,20例染色体G显带分析中,11例(55%)为复杂核型,8例(40%)为亚二倍体。11例患者接受染色体FISH检查结果显示,6例(54%)携带有17p13缺失,8例(73%)携带有t(14;16)、t(8;14)、t(11;14)、17p13缺失和13q14缺失中2种以上异常。全部患者总体中位生存时间为10.5个月,诊断时ECOG评分≤2分组患者中位生存时间为21.5个月,显著长于ECOG评分>2分组的1.2个月(P=0.017);接受新药(蛋白酶体抑制剂和/或免疫调节剂)治疗组患者中位生存时间为24.9个月,显著长于传统化疗组的10.5个月(P<0.001);在接受新药治疗的患者中,2种新药联合治疗组患者中位生存时间为30.9个月,显著长于新药单药治疗组的11.5个月(P=0.021)。遗传学异常对生存时间的影响差异无统计学意义。多因素统计分析显示,诊断时ECOG评分>2分是浆细胞白血病患者的独立预后不良因素。本研究中有2例患者行异基因造血干细胞移植,均于移植后6个月内死于肺部感染相关并发症。结论:在新药治疗条件下,诊断时ECOG评分是浆细胞白血病的独立预后危险因素,患者诊断后应尽早行多种新药联合治疗以进一步改善预后。肺部感染是异基因造血干细胞移植后患者死亡的常见原因。Objective:To evaluate the clinical characteristics,genetic abnormalities,treatment efficacy and prognostic factors in patients with plasma cell leukemia(PCL).Methods:30 patients diagnosed as PCL in our hospital from January 1993 to December 2019 were enrolled,and the clinical characteristics,laboratory findings,therapeutic regimes,and survival data of the patients were retrospectively analyzed.Results:The median age of the 30 patients was 56.5(28-80)years old,among them,25 patients were primary plasma cell leukemia,and 5 patients were secondary plasma cell leukemia.Complex karyotypes and subdiploids were most common in cytogenetic abnormalities.Among the 20 cases of chromosome G banding,11(55%)cases were complex karyotypes and 8(40%)cases were hypodiploid.Fluorescent in situ hybridization(FISH)test showed that among 11 cases,6 cases showed 17p13 deletion,8 cases showed at least two kinds of abnormalities,which including t(14;16),t(8;14),t(11;14),17p13 deletion,and 13q14 deletion.The median overall survival(OS)time was 10.5 months for all patients.The median OS time of the patients in ECOG score≤2 group was 21.5 months,which was significantly longer than those in the ECOG score>2 group(1.2 months)(P=0.017).The median OS time of the patients treated with novel agents(including proteasome inhibitor and/or immunomodulator)was 24.9 months,which was significantly longer than the patients treated with traditional chemotherapy group(10.5 months)(P<0.001).For the patients treated with novel agents,the median OS time of patients accepted two novel agents combination was 30.9 months,which was longer than those of single novel agent(11.5 months)(P=0.021).The effect of genetic abnormolity to the OS of the patients showed no statistical difference.Multivariate statistical analysis showed that ECOG score>2 was the independent prognostic factor of plasma cell leukemia patients.There were two patients underwent allogeneic hematopoietic stem cell transplantation in the study,but died due to the pulmonary infection within 6 month

关 键 词：浆细胞白血病 临床特征 遗传学异常 预后 

分 类 号：R733.7[医药卫生—肿瘤]