儿童间变性大细胞淋巴瘤并骨侵犯的临床研究  

Study on clinical features of childhood-anaplastic large cell lymphoma with bone involvement

作　　者：杨菁[1] 郑胡镛[1] 段彦龙[1] 金铃[1] 黄爽[1] 张梦[1] 周春菊[1] 张永红[1] 

机构地区：[1]首都医科大学附属北京儿童医院血液病中心,100045

出　　处：《中华实用儿科临床杂志》2014年第3期203-206,共4页Chinese Journal of Applied Clinical Pediatrics

基　　金：北京市卫生局首都医学发展科研基金（2007-1030）

摘　　要：目的 探讨间变性大细胞淋巴瘤（ALCL）并骨侵犯患儿的临床特征及其与预后的关系.方法 对2003年1月至2010年5月首都医科大学附属北京儿童医院收治的13例ALCL并骨侵犯患儿（骨侵犯组）的临床资料进行回顾性分析,并以同期收治的无骨侵犯的26例ALCL患儿作为对照（无骨侵犯组）,对2组患儿的临床特征及生存率进行统计学分析.结果 骨侵犯患儿占同时期收治的ALCL患儿的33.3％（13/39例）.其中2例为原发性骨淋巴瘤,占骨侵犯患者的15.4％（2/13例）,占同期全部ALCL患者的5.1％（2/39例）.误诊率为53.8％（7/13例）.多数患儿为多发骨侵犯,最易受累的区域是骨盆和脊柱.2组患儿年龄、性别、分期、病理亚型、肿瘤负荷等方面无统计学差异（P均＞0.05）.所有患儿只接受联合化疗,未行放疗.3年无事件生存率骨侵犯组为（84.6±10.0）％,无骨侵犯组为（76.9±8.3）％,差异无统计学意义（x2=0.506,P=0.307）.3年总体生存率骨侵犯组为（92.3±7.4）％,无骨侵犯组为（84.6±7.1）％,差异无统计学意义（x2=1.045,P=0.477）.患者停药前复查X线平片、CT和/或磁共振（MRI）等影像学检查,显示骨破坏完全修复者仅4例,未完全修复的9例患者中有3例同时行正电子发射计算机断层扫描（PZT）/CT检查,均未见高代谢灶.结论 骨侵犯不是ALCL患儿预后不良的相关因素,但临床特征和影像学表现特异性差,较易误诊,临床医师应加强对本病的认识.停药前多数患者骨破坏的影像学改变未完全恢复,故不能作为有残留病灶的依据.Objective To investigate the clinical features and prognostic value of childhood anaplastic large cell lymphoma（ALCL） with bone involvement.Methods The clinical features and prognosis of 13 ALCL patients with bone involvement and 26 patients without bone involvement admitted to Beijing Children＇s Hospital Affiliated to Capital Medical University from Jan.2003 to May 2010 were evaluated and compared retrospectively.Results Patients with bone involvement accounted for 33.3% （13/39 cases） of ALCL patients enrolled in the study.There were 2 primary bone lymphoma cases,accounting for 15.4% （2/13 cases） of the patients with bone involvement and 5.1% （2/39 cases） of all the ALCL patients enrolled in the study.The clinical misdiagnosis rate reached to 53.8% （7/13 cases）.Most tumors involved multiple sites and the most common sites of disease were the spine and the pelvis.The clinical features including age,stage,sex,visceral involvement,and cytomorphologic pattern were compared by statistical analysis between the 2 groups,there were no significant difference （all P 〉 0.05）.All the patients received combined chemotherapy without radiotherapy.They had a 3-year event-free survival （84.6 ± 10.0） %,compared with （76.9 ± 8.3）% for patients without bone involvement.There was no statistical difference （x2 =0.506,P =0.307）.And their 3-year overall survival was （92.3 ± 7.4） %,compared with （84.6 ± 7.1） % for patients without bone involvement,and there was no statistical difference either（x2 =1.045,P =0.477）.The sites of bone involvement were detected by X-ray,CT scan,and/or MRI before the end of chemotherapy,but the images did not recover completely in 9 cases,and 3 of the 9 cases underwent （18）F-FDG-PET/CT （PET/CT） scan at the same time,but all the outcomes were negative.Conclusions Bone involvement is not an unfavorable prognostic factor for ALCL patients.The specificity of clinical features and imaging of bone involvement is not remarkable in ALCL patients.Clinical

关 键 词：淋巴瘤 大细胞 间变性 儿童 骨 预后 

分 类 号：R733.1[医药卫生—肿瘤]