儿童霍奇金淋巴瘤HL-98方案临床报告

Clinical report on protocol HL-98 for childhood Hodgkin＇s Lymphoma

目的 获得在规范治疗下我国霍奇金淋巴瘤（HL）患儿的远期预后资料.方法 根据国外发达国家报告设计诊断与治疗方案,根据个体分期、肿瘤大小、有无全身症状分为低、中和高危3组（R1,R2,R3）,不同组别治疗强度不同,分别接受4、6、9个疗程短程化疗.根据对治疗的不同反应确定在化疗结束时是否给予15～20 Gy的受累野放疗.对1998年至2008年12月明确诊断的连续病例进行分析.采用Kaplan-Meier法计算全组生存率.结果 研究期间共纳入26例患儿.中位年龄94.5（30～179）个月.男20例,女6例.Ⅰ期3例,Ⅱ期4例,Ⅲ期9例,Ⅳ期10例.26例中24例有原发或转移性颈部淋巴结病变,12例存在纵隔病变,11例有脾脏多发性浸润,5例有B症状.进入R14例,R212例,R3 10例.25例完成所有治疗计划,1例Ⅱ期患儿未行最后1个疗程.,26例患儿中有8例在完成化疗计划时因增强CT提示有残留病灶可能而接受受累野放疗,7例总剂量为20～26 Gy,1例91个月患儿接受36 Gy.随访至2009年6月,26例患儿中有21例（80.76%）持续完全缓解（CCR）中.中位随访时间为31（10～120）个月.5例复发（Ⅲ期1例,Ⅳ期4例）.其中1例Ⅲ期患儿颈部原发部位复发、4例Ⅳ期患儿分别为全身、腹腔、胸腔和肝脏复发,平均复发时间为8（5～12）个月.Ⅰ期与Ⅱ期共7例患儿均在CCR中,CCR率为100% Ⅲ期9例患儿中1例复发,CCR率为89% Ⅳ期10例患儿中4例复发,CCR率为60%.预计5年总生存率为85.9%,5年无事件生存率为73.7%.有B症状者共5例,其中Ⅲ期1例,Ⅳ期4例,4例Ⅳ期患儿中3例复发.结论 该方案预计5年无事件生存率73.7%,提示方案有合理性 Ⅰ、Ⅱ期患儿无放疗者预后仍良好.

Objective To improve the long-term prognosis of childhood Hodgkin＇ s lymphoma （HL）by standard treatment protocol HL-98. Methods Patients were divided into low （ R1 ）, middle （ R2 ） and high-risk （R3 ） groups based on staging, tumor size and with or without B symptoms. Patients of R1 ,R2 and R3 groups were given 4, 6, and 9 courses of chemotherapy, respectively. Low dose radiotherapy to involved area was given to patients with residual disease at the end of chemotherapy. All patients diagnosed between 1998 and Dec. 2008 were enrolled. The software of SPSS 11.0 was used and the event free survival（EFS）was generated by Kaplan-Meier. Results There was a total of 26 patients with male 20 and female 6. The average age was 97 （30 to 179） months and median age 94.5 months. Three patients were in stage Ⅰ , 4 in stage Ⅱ , 9 in stage Ⅲ and 10 in stage Ⅳ. Of 26 patients, 24 were found with neck tumor, 12 with mediastinum tumor, 1 1 with spleen infiltration and 5 with B symptom. Four patients were allocated into R1 group, 12R2 group and 10 R3 group. Eight of 26 with residual disease received radiotherapy, 7 received 20 -26 Gy and 1 received 36 Gy. To Jun 2009, 21 （80.76%） of them kept in complete remission（CR） at 10 to 120 months follow-up （average 36 months, and median 31 months）. Five cases relapsed （1 of stage Ⅲ and 4 of stage Ⅳ ） within 5 to 12 months. Three out of 4 in stage Ⅳ with B symptom relapsed. The estimated 5-year overall survival （OS） was 85.9% and EFS was 73.7%. Conclusion The estimated 5-year EFS indicated that protocol HL-98 is reasonable good. Patients of stage Ⅰ and Ⅱ can obtain a good prognosis without radiotherapy.