含依托泊苷方案诱导治疗成人Still病相关噬血细胞综合征临床分析  

Clinical study on the effect of etoposide-containing induction regimen for adult-onset Still′s disease associated hemophagocytic syndrome

作　　者：贺凌博 金志丽[1] 刘孟涵 吴林[1] 王昭[1] 王旖旎[1] He Lingbo;Jin Zhili;Liu Menghan;Wu Lin;Wang Zhao;Wang Yini(Department of Hematology,Beijing Friendship Hospital of Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院血液科,北京100050

出　　处：《中华医学杂志》2021年第29期2322-2327,共6页National Medical Journal of China

基　　金：北京市医院管理局扬帆计划临床技术创新项目(XMLX201823);国家自然科学基金(81871633);北京市自然科学基金(7181003);北京市医院管理局登峰团队人才项目(DFL20180101)。

摘　　要：目的探讨含依托泊苷(VP-16)方案治疗成人Still病相关噬血细胞综合征(AOSD-HLH)的疗效。方法回顾性收集2014年12月至2019年12月于首都医科大学附属北京友谊医院诊治的AOSD-HLH患者43例的临床特点、实验室指标、治疗和预后资料,其中,男7例,女36例,年龄30(24,40)岁。根据初始治疗方案中是否包含VP-16进行分组,初始治疗方案不含VP-16者为组1(n=31),组1中对初始治疗无反应的患者,采用含有VP-16的方案进行二次诱导缓解;初始治疗中含VP-16者为组2(n=12)。比较2组患者初始治疗的效果。将噬血细胞综合征获得缓解的患者,依据是否应用含有VP-16的治疗方案分为组a(未应用,n=6)和组b(应用,n=33),比较2组患者的实验室指标。结果组1患者的总体应答率(ORR)(6/31比11/12)及完全缓解率(CRR)(1/31比5/12)显著低于组2患者(均P<0.05);组1中对初始治疗无应答的患者,使用含VP-16的治疗方案二次诱导缓解,ORR达22/24。组b患者较组a患者具有更高的中性粒细胞计数[5.6(3.4,9.3)×10^(9)/L比3.9(2.3,4.7)×10^(9)/L]、自然杀伤细胞活性[16.3(14.2,17.5)%比13.1(12.2,13.8)%](均P<0.05),血红蛋白[103.0(97.0,109.5)g/L比91.5(70.0,118.0)g/L]及血小板计数[(212.2±74.2)×10^(9)/L比(226.0±114.9)×10^(9)/L]差异无统计学意义(均P>0.05)。结论HLH的缓解状态对患者的预后存在影响,初始治疗中应用VP-16可以显著提高AOSD-HLH患者的ORR及CRR,VP-16的应用并未引起患者骨髓抑制。Objective To investigate the efficacy of an etoposide-containing regimen in the treatment of adult-onset Still′s disease related hemophagocytic syndrome(AOSD-HLH).Methods This study adopted the method of retrospective analysis to collect clinical data of 43 AOSD-HLH patients,including the clinical characteristics,laboratory indexes,treatment regimen and prognosis.There were 7 males and 36 females,aged 24-40 years,with a median age of 30.All of them were diagnosed and treated in Beijing Friendship Hospital of Capital Medical University from December 2014 to December 2019.According to whether or not etoposide(VP-16)was included in the initial therapy,patients were divided into group 1(VP-16 was not administrated in the initial treatment,n=31)and group 2(the initial treatment included etoposide,n=12).Patients in group 1 who did not respond to the initial treatment were retreated with VP-16-containing regimen,and the effect of initial treatment was compared between the 2 groups.Similarly,according to whether the VP-16-containing regimen was applied or not,patients achieving remission of HLH were divided into group a(not applied,n=6)and group b(applied,n=33),and the laboratory indicators of the two groups were compared.Results The overall response rate(ORR,6/31 vs 11/12)and complete response rate(CRR,1/31 vs 5/12)of patients in group 1 were significantly lower than those in group 2(both P<0.05).Patients in group 1 who did not respond to the initial treatment were retreated with a VP-16-containing regimen,and we found that the ORR reached 22/24.Among patients in remission,the natural killer cell activity[16.3(14.2,17.5)%vs 13.1(12.2,13.8)%]and granulocyte counts[5.6(3.4,9.3)×10^(9)/L vs 3.9(2.3,4.7)×10^(9)/L]of patients was significantly higher in group B than that in group A(both P<0.05).There was no statistically significant difference in haemoglobin[103.0(97.0,109.5)g/L vs 91.5(70.0,118.0)g/L]and platelet counts[(212.2±74.2)×10^(9)/L vs(226.0±114.9)×10^(9)/L]between the two groups(both P>0.05).Conclusion The

关 键 词：Still病 成年型 噬血细胞综合征 依托泊苷 

分 类 号：R551.2[医药卫生—血液循环系统疾病]