原发系统型间变性大细胞淋巴瘤40例临床特征及预后分析  

Clinical characteristics and prognostic factors of 40 cases of primary systemic anaplastic large cell lymphoma

作　　者：陈英坤 俞文娟[1] 刘辉[1] 韦菊英[1] 钱文斌[1] 金洁[1] Chen Yingkun;Yu Wenjuan;Liu Hui;Wei Juying;Qian Wenbin;Jin Jie(The First Affiliated Hospital of Zhejiang University,Hangzhou 310003,China;The First People’s Hospital of Xiaoshan District,Hangzhou 311200,China)

机构地区：[1]浙江大学医学院附属第一医院,杭州310003 [2]杭州市萧山区第一人民医院,311200

出　　处：《中华血液学杂志》2020年第3期222-227,共6页Chinese Journal of Hematology

摘　　要：目的分析原发系统型间变性大细胞淋巴瘤(ALCL)的临床特征及预后因素。方法回顾性分析2013年1月至2018年12月期间在浙江大学医学院附属第一医院住院治疗的40例ALCL患者的临床资料,总结其临床特征并分析其预后相关因素。结果①40例患者中男29例,女11例,中位年龄41(14~67)岁,Ann Arbor分期Ⅲ~Ⅳ期36例(90.0%),IPI评分中高危、高危23例(57.5%);25例(62.5%)有B症状,发热最为常见,其次为消瘦、盗汗;38例(95.0%)有结外侵犯;25例(62.5%)LDH升高;25例(62.5%)Ki-67≥80%;ALK阳性22例(55.0%),中位年龄29(14~67)岁,ALK阴性18例(45.0%),中位年龄51.5(19~67)岁,年龄差异有统计学意义(P=0.003)。②40例患者均接受化疗,采用CHOP(环磷酰胺+多柔比星+长春地辛+泼尼松)方案18例,ECHOP(依托泊苷+CHOP)方案12例,其他方案10例;26例(65.0%)获得完全缓解(CR),ALK阴性(P=0.029,OR=13.458)和Ki-67≥80%(P=0.04,OR=14.453)是CR的独立影响因素,ECHOP方案的CR率高于CHOP方案[83.3%(10/12)对38.9%(7/18),P=0.026]。③LDH水平、IPI评分、ALK表达情况以及化疗方案的选择对无进展生存(PFS)和总生存(OS)影响有统计学意义(P<0.05)。结论ALCL发病男性居多,青少年至中老年均有发病,ALK阳性中年轻患者居多,诊断时临床分期晚,多数患者有结外侵犯,半数以上患者有B症状、LDH升高、Ki-67高表达;ALK表达与否、Ki-67表达水平以及化疗方案的选择可影响疗效,LDH水平、IPI评分、ALK表达与否以及化疗方案的选择可影响PFS和OS;ECHOP是较优的化疗方案,可改善预后。Objective To analyze the clinical features and prognostic factors of primary systemic anaplastic large cell lymphoma(ALCL).Methods 40 ALCL cases treated in the First Affiliated Hospital of Zhejiang University from January 2013 to December 2018 were retrospectively analyzed.Results①With a median age of 41(14-67)years,there were 29 males and 11 females,36 patients(90.0%)had Ann Arbor stageⅢ-Ⅳtumors,23 patients(57.5%)were in high-intermediate or high international prognostic index(IPI)risk group.25 patients(62.5%)had B symptoms,such as fever,emaciation and night sweat.38 patients(95.0%)had extranodal invasion,25 patients(62.5%)had higher LDH level,and 25 patients(62.5%)had high expression of Ki-67(80%or more).With 22 ALK+patients(55.0%)and 18 ALK-patients(45.0%),there was a significantly difference in the median age of the two groups[29(14-67)years old vs 51.5(19-67)years old,P=0.003].②All patients received chemotherapy,18 cases were treated with CHOP(cyclophosphamide,doxorubicin,vindesine,prednisone),12 cases with ECHOP(cyclophosphamide,doxorubicin,vindesine,prednisone,etoposide),10 cases with other treatments and 26 patients(65.0%)obtained complete remission(CR).ALK-(P=0.029,OR=13.458)and Ki-67 expression of 80%or more(P=0.04,OR=14.453)were independent factors of CR rate,the CR rate of ECHOP chemotherapy was higher than CHOP chemotherapy(P=0.026).③LDH level,IPI score,ALK expression and chemotherapy regimen had significantly effect on progression free survival(PFS)and overall survival(OS)(P<0.05).Conclusion The study shows that primary systemic ALCL usually occurs in males,the average age of ALK+patients were younger than ALK-patients.Most patients are in stageⅢ-Ⅳwith extranodal invasion,more than half of the patients have B symptoms,elevated LDH,and high expression of Ki-67.The expression level of Ki-67,ALK expression,and chemotherapy regimen have prognostic value for CR rate,the LDH level,IPI score,ALK expression and chemotherapy regimen for PFS and OS.ECHOP is a better choice with improved prognosi

关 键 词：间变性大细胞淋巴瘤 间变性淋巴瘤激酶 临床特征 预后因素 

分 类 号：R733[医药卫生—肿瘤]