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

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。

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

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 high-intensity 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 treatment-related toxicities.