改良NHL-BFM-90方案治疗儿童青少年淋巴母细胞淋巴瘤患者远期疗效分析

Outcomes of modified NHL-BFM-90 protocol for children and adolescents with lymphoblastic lymphoma

目的 评估改良NHL-BFM-90方案治疗儿童青少年淋巴母细胞淋巴瘤（LBL）患者的远期疗效.方法 以1998年3月至2010年12月收治的107例初治LBL患者（年龄≤18岁）为研究对象,根据临床分期和早期诱导化疗疗效将患者分为低危、中危和高危组,采用改良NHL-BFM-90方案化疗.不同分组患者采用不同强度化疗.总疗程2年.结果 107例患者中,男79例（73.8％）,女28例（26.2％）,中位年龄10（2.5～18）岁.Ⅰ/Ⅱ期者6例（5.6％）,Ⅲ/Ⅳ期者101例（94.4％）.低危组6例（5.6％）,中危组76例（71.0％）,高危组25例（23.4％）.B-LBL 26例（24.3％）,T-LBL 81例（75.7％）.中位随访时间61（1～186）个月.24例患者死亡.5年无事件生存率和总生存率分别为：全组75.5％和77.8％;低危组100.0％和100.0％;中危组84.4％和87.5％;高危组44.0％和44.0％; T-LBL患者72.0％和73.5％;B-LBL患者86.4％和88.5％.骨髓抑制是主要不良反应,需要积极处理.结论 改良NHL-BFM-90方案对低危和中危儿童青少年LBL患者有很好的疗效,T-LBL和B-LBL患者疗效相似.高危患者疗效差,需要探讨新的治疗方法.

Objective To evaluate the long-term survival of children and adolescents with lymphoblastic lymphoma （LBL） treated by a modified NHL-BFM-90 protocol.Methods From March 1998 to November 2010,107 untreated patients with LBL （age 〈 18 years） were enrolled and stratified into three groups （R1,R2 and R3）,according to the stage of disease and response to induction chemotherapy.All patients received different intensive chemotherapy regimens based on a modified NHL-BFM-90 protocol.Total treatment duration was 2 years.Results Of the 107 patients,79 were boys and 28 were girls,with a median age of 10 years （range 2.5-18 years）.Six patients （5.6%） were stage Ⅰ/Ⅱ,101 （94.4%） stage Ⅲ/Ⅳ.The R1,R2 and R3 groups accounted for 5.6%,71.0% and 23.4%,respectively.75.7% of the patients had T-LBL,and 24.3% was B-LBL.At a median follow-up duration of 60 months （range 1-186 months）,24 patients died.The 5-year event-free survival （EFS） and overall survival （OS） were 75.5% and 77.8 % for all patients,100.0% and 100.0% for group R1,84.5% and 87.5 % for R2,44.0% and 44.0% for R3,72% and 73.5% for T-LBL,86.4% and 88.5% for B-LBL,respectively.Myleosuppression was the major toxicity and need aggressive management.Conclusion The modified NHL-BFM-90 protocol is an effective therapy for children and adolescents with LBL in low and intermediate risk.T-LBL had the similar outcomes as B-LBL did.The patients in high-risk group had a poor survival and new protocols are needed.