摘要
目的总结分析儿童间变性大细胞淋巴瘤(anaplastic large-cell lymphoma,ALCL)治疗方案的远期疗效及影响预后的因素。方法回顾性分析2002年1月-2010年4月在我院诊断并接受治疗和随访的20例ALCL患儿,采用本院制定的B-NHL 2001方案进行危险度分组和治疗,采用SPSS软件对数据进行处理。结果 ALCL患儿20例,男9例,女11例,诊断时中位年龄8.4(1.9～15.4)岁,>10岁者7例。诊断时Ⅱ期、Ⅲ期和Ⅳ期患儿分别有1例、11例和8例。8例原发于颈部淋巴结,而纵隔、腹腔、腹股沟和腋窝等部位各2例,右腰大肌和左上臂肌各1例,髋骨和右肱骨各1例。15例发病时有B症状,6例ECT显示有骨骼侵润,2例骨髓穿刺见肿瘤细胞,初诊时血清乳酸脱氢酶和铁蛋白增高1倍以上者各6例。病理分析显示17例为T细胞型,16/18例免疫组化间变性大细胞淋巴瘤激酶(ALK)阳性。所有患儿均接受了R3组治疗,有2例初诊为横纹肌肉瘤,其中1例给予AVCP方案一疗程后明确诊断转入R3,1例Ⅱ期患儿接受R2期治疗未缓解转入R3,另有1例初诊T-NHL,以PVDL方案治疗一疗程转入R3。5年无事件生存率和总生存率分别为(69.3±10.5)%和(74.0±10.1)%。单因素分析发现年龄>10岁组患儿复发风险更大,而性别、病理类型、分期、原发部位、是否有骨骼或骨髓侵润、血清乳酸脱氢酶和铁蛋白水平等,对复发风险在统计学上无显著影响。结论儿童ALCL的临床表现多样,其疗效受年龄影响,目前按照B-NHL2001治疗方案能取得较好的远期疗效。
Objective To analyze the clinical characteristics and prognosis of children with anaplastic large-cell lymphoma（ALCL）.Methods Twenty children with newly diagnosed ALCL were enrolled in the protocol of B-NHL 2001 from Jan of 2002 to Apr of 2010.Their clinical characteristics,therapy and outcome were analyzed.Results There were 9 males among the 20 children.The mean age at diagnosis was（8.7±3.4）（1.9 to 15.4） years old with the median age of 8.4 years old.Seven children were older than 10 years old.One patient was in stage II,11 in stage III and 8 in stage IV.The original tumor was found in cervical lymph node（8 cases）,mediastinum（2 cases）,abdomen（2 cases）,groin（2 cases）,axillary lymph node（2 cases）,muscle（2 cases） and bone（2 cases）.Fifteen patients had B symptoms.ECT showed that 6 children had bone involvement and 2 patients had bone marrow involvement.Elevated LDH and serum ferritin were respectively presented in 6 children.Pathology analysis showed that 17 were positive for T-cell markers and 16/18 patients were positive for ALK.All the patients were allocated into high risk group（R3）.The estimated 5-year EFS and OS were（69.3±10.5）% and（74.0±10.1）%,respectively.According to univariate analyses,patients with more than 10 years old had higher risk for recurrence.Sex,immunophenotype,original tumor,stage,bone or marrow involvement,elevated LDH and SF were not associated with the recurrent risk.Conclusions B-NHL 2001 protocol was reasonable for children with ALCL and worth to enroll more patients.
出处
《中国小儿血液与肿瘤杂志》
CAS
2012年第1期13-16,共4页
Journal of China Pediatric Blood and Cancer
关键词
儿童
间变性大细胞淋巴瘤
临床疗效
无事件生存
Child
Anaplastic large-cell lymphoma
Therapeutic effect
Event tree survlvat
