青少年或儿童原发系统性间变大细胞淋巴瘤CHOP为主化疗±放疗的远期疗效

Long-term efficacy of CHOP-based chemotherapy alone or combined with radiotherapy for adolescent/childhood primary systemic anaplastic large-cell lymphoma

目的 分析青少年儿童原发系统性间变大细胞淋巴瘤（ALCL）患者接受CHOP方案化疗±受累野放疗的疗效.方法 回顾分析本院1998-2010年收治的28例青少年、儿童ALCL患者资料.Ⅰ、Ⅱ期12例中单纯化疗2例、综合治疗10例,Ⅲ、Ⅳ期16例中单纯化疗14例、综合治疗2例.CHOP方案15例、CHOP联合其他高强度化疗13例.化疗周期数3～17个（中位数6个）.放疗多为受累野照射,剂量39.6～ 50.0 Gy（中位数45 Gy）.结果 全组患者首程疗后达CR者25例（89％）,3例病变进展.中位随访时间45.3个月.全组5年无事件生存率为80％,5年OS为93％.疗终达CR者5年OS为100％,而未达CR者无5年OS（P=0.000）.≥2个结外器官受侵者5年无事件生存率为38％,而＜2个结外器官受侵者的为85％ （P =0.010）.结论 青少年、儿童原发系统性ALCL按成人方案治疗效果满意,但还需要长期随访.

Objective To investigate the long-term efficacy of CHOP （cyclophosphamide,doxorubicin,vincristine,and prednisone）-based chemotherapy alone or combined with regional radiotherapy for adolescent/childhood primary systemic anaplastic large-cell lymphoma （ALCL）.Methods A retrospective analysis was performed on the medical records of 28 patients not older than 21 years with a confirmed diagnosis of primary systemic ALCL,who were admitted to our hospital from January 1998 to December 2010.Of 12 stage Ⅰ / Ⅱ patients,2 received chemotherapy alone,and 10 received chemotherapy and radiotherapy;of 16 stage Ⅲ/Ⅳ patients,14 received chemotherapy alone,and 2 received chemotherapy and radiotherapy.CHOP regimen was adopted in 15 patients,and CHOP regimen combined with other highintensity regimens was adopted in 13 patients.There were 3-17 chemotherapy cycles （median：6 cycles）.Radiotherapy was performed mainly by involved field radiation,with a dose of 39.6-50.0 Gy （median：45 Gy）.Results After the first course of treatment,25 （89%） of all patients achieved a complete remission （CR）,and 3 patients experienced disease progression.With a median follow-up of 45.3 months,the 5-year event-free survival （EFS） and overall survival （OS） for all patients were 80% and 93%,respectively.The 5-year OS was 100% for patients achieving a CR after treatment,versus 0% for those not achieving a CR （P =0.000）.The 5-year EFS was 38 % for patients with involvement of ≥ 2 extranodal organs,versus 85 % for those with involvement of 〈 2 extranodal organs （P =0.010）.Conclusions CHOP chemotherapy combined with regional radiotherapy （for patients with localized disease） is an effective treatment for adolescent/childhood primary systemic ALCL,but long-term follow-up is needed to investigate treatmentrelated toxicities.