改良B-NHL-BFM-90方案治疗儿童青少年伯基特淋巴瘤的疗效分析

Efficacy of Modified B-NHL-BFM-90 Protocol on Burkitt's Lymphoma in Chinese Children and Adolescents

背景与目的:伯基特淋巴瘤是高度恶性非霍奇金淋巴瘤,进展快,常伴骨髓和中枢侵犯,死亡率高。CHOP方案疗效差,生存率低。伯基特淋巴瘤的最佳化疗方案仍需要积极探讨。本研究总结中山大学肿瘤防治中心近年来采用改良B-NHL-BFM-90方案治疗儿童青少年伯基特淋巴瘤的疗效和生存率。方法:从1999年10月至2006年11月,31例20岁以下经病理确诊的伯基特淋巴瘤患者入组。年龄1.5～20岁,中位年龄5岁;男性20例(64.5%),女性11例(35.5%)。临床分期(StJude分期):Ⅰ期1例(3.2%),Ⅱ期6例(19.4%),Ⅲ期8例(25.8%),Ⅳ期16例(51.6%),Ⅲ/Ⅳ期患者占77.4%。根据临床分期、治疗反应和LDH水平,将患者分为低危组、中危组和高危组。采用改良B-NHL-BFM-90方案治疗,药物包括cyclophosphamide、vincristine、ifosfamide、etoposide、adriamycin、HD-methotrexate、vindesine、dexamethasone、cytarabine/HD-cytarabine和鞘内注射。结果:31例患者中1例于诱导前期死于肿瘤溶解综合征。30例可评价疗效。30例中25例(83.3%)完全缓解,3例(10.0%)部分缓解,2例(6.7%)进展。1例复发。治疗期间大部分患者发生Ⅲ/Ⅳ度骨髓抑制,经积极对症支持治疗可恢复,不影响下一疗程治疗。中位随访33个月(3～98个月),全组3年无事件生存率86.0%;Ⅰ/Ⅱ期100%,Ⅲ/Ⅳ期82.1%;低危组100%,中危组92.0%,高危组70.0%。结论:改良B-NHL-BFM-90方案可明显改善儿童青少年伯基特淋巴瘤的疗效和生存率,毒性可耐受,但需要在有经验的肿瘤中心和血液科中应用。

BACKGROUND ＆ OBJECTIVE： Burkitt＇s lymphoma is an aggressive non-Hodgkin＇s lymphoma （NHL） and often involves bone marrow and central nerve system. The efficacy of CHOP regimen on Burkitt＇s lymphoma is poor. The optimal chemotherapy regimen needs to be investigated. This study was to evaluate the efficacy of modified B-NHL-BFM-90 protocol on Burkitt＇s tymphoma in children and adolescents, and observe the survival status. METHODS： From Oct. 1999 to Nov. 2006, 31 untreated Burkitt＇s lymphoma patients aged less than 20 were enrolled. The median age of these patients was 5 （range, 1.5-20 years old）. Of the 31 patients, 20 （64.5%） were male, 11 （35.5%） were female. According to St Jude staging system, 1 （3.2%） was at stage Ⅰ , 6 （19.4%） at stage Ⅱ, 8 （25.8%） at stage Ⅲ , 16 （51.6%） at stage Ⅳ ; 24 （77.4%） were at stage Ⅲ/Ⅳ. According to clinical stage, lactate dehydrogenase （LDH） level and treatment response, these patients were divided into low, moderate and high risk groups. They received modified B-NHL-BFM-90 protocol, cytotoxic drugs such as cyclophosphamide, vincristine, ifosfamide, etoposide, adriamycin, HD- methotrexate, vindesin, dexamethasone, cytarabinec/HD-cytarabine and intrathecal injection. RESULTS： One patient died of tumor lysis syndrome during prophase. The efficacy was evaluable in 30 patients. Of the 30 patients, 25 （83.3%） achieved completeremission （CR）, 3 （10.0%） achieved partial remission （PR）, 2 （6.7%） had progressive disease （PD）; 1 had tumor relapse. Grade 3-4 myelosuppression occurred in most patients and were recovered by active support care and did not affect next course of chemotherapy. At a median follow-up of 33 months （range, 3-98 months）, the 3-year event-free survival （EFS） rate was 86.0% for all patients, with 100% for stage Ⅰ / Ⅱ patients and 82.1% for stage Ⅲ/Ⅳ patients, 100% for low risk group, 92.0% for moderate risk group, and 70.0% for high risk group. CONCLUSIONS： Modified B-NHL-BFM-90 protocol can improve the responses and survival of Burkitt＇s lymphoma in Chinese children and adolescents, with tolerable toxicity. It should be used in the experienced cancer center and hematological unit.