CAJ | 학술논문

目的研究急性成熟B淋巴细胞性白血病(B-ALL)儿童的治疗,分析治疗方案的合理性及疗效.方法将部分符合B-ALL诊断标准病例定义为“成熟B淋巴细胞性白血病/淋巴瘤可能”(mature B-ALL/NHLp),制定入组标准及治疗方案.选择2003年2月至2012年12月在上海交通大学医学院附属上海儿童医学中心(SCMC)诊断并接受治疗和随访的mature B-ALL/NHLp患儿15例,采用本院制定的SCMC-mature B-ALL/NHLp-2003方案进行治疗,对患儿治疗过程进行回顾性评估分析.采用SPSS 21.0软件对数据进行处理.结果 15例患儿中诊断时年龄8.7岁(1岁5个月～14岁4个月).其中4例患儿以局部肿块为主要临床表现,包括颌面部肿块、颈部肿块和腹部肿块;余10例均有发热、面色苍白等全身表现.免疫分型示:膜免疫球蛋白M、末端脱氧核苷酸转移酶、Cμ、CD1o、CD19、cCD79a不同程度和比例表达.随访至2013年11月30日,无事件生存者中位随访时间为80个月(39 ～128个月),5年无事生存率为(80.0±10.3)％.单因素分析显示,乳酸脱氢酶＞4倍正常值、血清铁蛋白＞2倍正常值及无微小残留病标记为预后不良因素(x2=5.49、4.89、5.49,P均＜0.05).结论 SCMC-mature B-ALL/NHLp-2003方案治疗部分儿童mature B-ALL有合理性和临床现实意义,可进一步扩大样本进行临床研究.
목적 연구급성성숙B림파세포성백혈병(B-ALL)인동적치료,분석치료방안적합이성급료효.방법 장부분부합B-ALL진단표준병례정의위“성숙B림파세포성백혈병/림파류가능”(mature B-ALL/NHLp),제정입조표준급치료방안.선택2003년2월지2012년12월재상해교통대학의학원부속상해인동의학중심(SCMC)진단병접수치료화수방적mature B-ALL/NHLp환인15례,채용본원제정적SCMC-mature B-ALL/NHLp-2003방안진행치료,대환인치료과정진행회고성평고분석.채용SPSS 21.0연건대수거진행처리.결과 15례환인중진단시년령8.7세(1세5개월～14세4개월).기중4례환인이국부종괴위주요림상표현,포괄합면부종괴、경부종괴화복부종괴;여10례균유발열、면색창백등전신표현.면역분형시:막면역구단백M、말단탈양핵감산전이매、Cμ、CD1o、CD19、cCD79a불동정도화비례표체.수방지2013년11월30일,무사건생존자중위수방시간위80개월(39 ～128개월),5년무사생존솔위(80.0±10.3)％.단인소분석현시,유산탈경매＞4배정상치、혈청철단백＞2배정상치급무미소잔류병표기위예후불량인소(x2=5.49、4.89、5.49,P균＜0.05).결론 SCMC-mature B-ALL/NHLp-2003방안치료부분인동mature B-ALL유합이성화림상현실의의,가진일보확대양본진행림상연구.
Objective To evaluate the outcomes of mature B-cell acute lymphoblastic leukemia(mature B-ALL) and to assess the safety and efficacy of the treatment protocol.Methods From February of 2003 to December of 2012,15 children were diagnosed as mature B-cell acute lymphoblastic leukemia/lymphoma possible (mature B-ALL/NHLp) in Shanghai Children's Medical Center(SCMC) were enrolled,and they were treated with SCMC-mature B-ALL/NHLp-2003 protocol.All of the clinical characteristics,therapeutic effects and long-term outcomes were analyzed.The statistical data were processed by SPSS 21.0.Results The median age on diagnosis was 8.7 years (1 year and 5 months to 14 years and 4 months).Among them,4 cases presented with local mass including maxillofacial tumors,neck and abdominal mass.The others had systemic manifestations such as fever and pale face.These neoplastic cells retained the expressions of surface membrane immunoglobulin M,terminal deoxynucleotidyl transferase,Cμ,CD10,CD19,cCD79 a differently.Follow-up was updated to November 30,2013.The median follow-up period was 80 months (39-128 months).Theestimated 5-year event free survival rate was (80.0 ± 10.3) ％.According to univariate analysis,increased lactate dehydrogenase level (＞ 4-times the normal value),increased serum ferritin level (＞ 2-times the normal value),no small residual disease markers were indepen-dent poor prognostic factors(x2 =5.49,4.89,5.49,all P ＜ 0.05).Conclusions SCMC-mature B-NHL/ALLp-2003 protocol is feasible and safe for children with mature B-ALL/NHLp,but more sample cases need to be investigated.