89例髓系肉瘤临床特征及预后因素分析  

Clinical features and prognostic factors of 89 cases of myeloid sarcoma

作　　者：汤平[1] 董振坤 郭荣[1] 王海琼 卢润青 谢新生[1] 孙慧[1] 孙玲[1] 万鼎铭[1] 刘延方[1] 姜中兴[1] Tang Ping;Dong Zhenkun;Guo Rong;Wang Haiqiong;Lu Runqing;Xie Xinsheng;Sun hui;Sun ling;Wan Dingming;Liu Yanfang;Jiang Zhongxing(Department of Hematology,The First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院血液内科,450052

出　　处：《中华器官移植杂志》2021年第3期173-176,共4页Chinese Journal of Organ Transplantation

基　　金：国家自然科学基金(81070445);河南省自然科学基金(182300410301);河南省医学科技攻关计划(201403029)。

摘　　要：目的分析研究髓系肉瘤的临床特征、治疗及预后。方法回顾性分析2010年1月至2019年5月郑州大学第一附属医院诊治的89例髓系肉瘤病例资料,将89例髓系肉瘤患者按疾病分型不同分为原发性髓系肉瘤组(40例)、髓系肉瘤合并髓内疾病组(36例)和白血病髓外复发标志的髓系肉瘤(13例)。统计其年龄、性别、发病部位、类型、合并疾病、有无淋巴特征、疾病缓解状态等临床特征及是否化疗和移植、移植后是否用去甲基化药物(HMA)维持等对1年存活率的影响。结果89例髓系肉瘤中21例进行染色体核型分析及二代基因测序,8例进行异基因全相合造血干细胞移植(allo-HSCT)。原发性髓系肉瘤、髓系肉瘤并髓内疾病、白血病缓解后以髓系肉瘤复发的1年总体存活率(OS)分别为16.0%、37.5%、36.9%,差异有统计学意义(P=0.013)。局部治疗(手术切除、鞘内注射、局部放疗)、化疗联合局部治疗、化疗联合allo-HSCT的1年OS分别为:0、28.1%、72.9%,差异有统计学意义(P=0.003)。两疗程治疗后完全缓解和未完全缓解患者的1年存活率分别为34.9%、10.0%(P=0.008)。50%(4/8)髓系肉瘤受者移植后1年内复发,且生存期短。3例移植后受者应用地西他滨维持治疗,目前均长期存活。结论化疗联合allo-HSCT是髓系肉瘤的有效治疗手段,移植后应用地西他滨维持治疗可能延长无复发生存时间,但尚需扩大样本量进一步临床验证。Objective To explore the clinical characteristics,treatment and prognosis of myeloid sarcoma(MS).Methods From January 2010 to May 2019,clinical data were reviewed for 89 MS cases.Age,gender,site of onset,type,comorbid diseases,lymphatic characteristics and disease remission status were analyzed.And 1-year survival rates were explored for different treatments including whether or not chemotherapy,transplantation and using hypomethylated drugs(HMAs)for maintenance after transplantation.Results Among them,21 cases had the data of chromosome karyotypic analysis and next generation sequencing and 8 patients underwent allogeneic hematopoietic stem cell transplantation(allo-HSCT).The 1-year overall survival rates(OS)of primary MS,MS with intramedullary disease and MS relapse after leukemic remission were 16.0%,37.5%and 36.9%respectively(P=0.013).The 1-year OS of local treatment(surgical resection,intrathecal injection and local radiotherapy),chemotherapy plus local treatment and chemotherapy plus allo-HSCT was 0,28.1%and 72.9%respectively(P=0.003).After two courses of treatment,the 1-year OS of patients with complete and incomplete remissions were 34.9%and 10.0%respectively(P=0.008).Half(4/8)MS patients relapsed within 1 year after transplantation and had a short survival.Three patients received decitabine after HSCT and all of them survived for a long time.Conclusions Chemotherapy plus HSCT is efficacious for MS.Decitabine maintenance treatment after transplantation may prolong recurrence-free survival.However,a larger sample size is required for further clinical verifications.

关 键 词：髓系肉瘤 造血干细胞移植 地西他滨 

分 类 号：R733.3[医药卫生—肿瘤]