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儿童和青少年非霍奇金淋巴瘤的治疗(基于NHL柏林-法兰克福-明斯特90方案的结果)

Treatment of children and adolescents with non-Hodgkin's lymphoma (results based on the NHL Berlin-Frankfurt-Münster 90 protocols).

作者信息

Kavan P, Kabicková E, Gajdos P, Koutecký J, Smelhaus V, Stanková J, McClain K L

机构信息

Department of Pediatric Oncology, University Hospital Motol, Prague, Czech Republic.

出版信息

Cas Lek Cesk. 1999 Jan 18;138(2):40-6.

Abstract

BACKGROUND

To determine the feasibility and results of treating children with non-Hodgkin's lymphomas (NHL) according to very intensive protocols based on the German Berlin Frankfurt Münster NHL 90 study.

METHODS AND RESULTS

From 1991 until 1995 eighty two patients less than 18 years of age with NHL were admitted to our department. Sixty three of them were eligible for the study. The entire group consisted of 43 males and 20 females (ratio 2.1:1). Median age was 10 2/12 years. Eleven had stage I disease, 4 stage II, 29 stage III and 19 stage IV disease. Histologies represented were: large cell lymphoma 22, lymphoblastic lymphoma 19, and Burkitt lymphoma 10 patients. In 12 cases the immunophenotype was not further classified as to B-cell or T-cell subtype. Patients were stratified into the therapy groups "B" or "non B" according to histopathology, clinical stage and LDH level. Therapy for the B group consisted of 2, 4 or 6 courses of intensive 5 day pulses of 6 drugs. Patients in the non B group received the protocol for acute lymphoblastic leukemia including reinduction and CNS irradiation for advanced stages. At a median follow-up of 35 months the probability of event free survival (pEFS) at 5 years 70% and overall survival 73% for entire group. For therapy group B pEFS was 76%. The non B therapy group had a pEFS 60% (p = 0.22). There was a significantly better outcome for children classified as stage I and II. There was no statistical difference between stage III and IV. Treatment results were comparable between NHL subtypes, except for large cell lymphomas, which did significantly better (pEFS 90%).

CONCLUSIONS

The use of protocols based on BFM 90 study in the Czech Republic was feasible. The pEFS are approximately 10% lower than the German study but comparable to some other studies. Outcome for large cell lymphomas was excellent. Reduction of treatment related complication and mortality rate as well as more precise classification are required.

摘要

背景

根据基于德国柏林-法兰克福-明斯特非霍奇金淋巴瘤(NHL)90研究的高强度方案,确定治疗儿童非霍奇金淋巴瘤(NHL)的可行性及结果。

方法与结果

1991年至1995年,82例18岁以下的NHL患者入住我科。其中63例符合研究条件。整个组包括43名男性和20名女性(比例为2.1:1)。中位年龄为10又2/12岁。11例为Ⅰ期疾病,4例为Ⅱ期,29例为Ⅲ期,19例为Ⅳ期疾病。组织学类型包括:大细胞淋巴瘤22例,淋巴母细胞淋巴瘤19例,伯基特淋巴瘤10例。12例的免疫表型未进一步分为B细胞或T细胞亚型。根据组织病理学、临床分期和乳酸脱氢酶水平,将患者分层到治疗组“B”或“非B”。B组的治疗包括2、4或6个疗程的6种药物强化5天脉冲治疗。非B组患者接受急性淋巴细胞白血病方案,包括晚期患者的再诱导和中枢神经系统照射。中位随访35个月时,整个组5年无事件生存率(pEFS)为70%,总生存率为73%。治疗组B的pEFS为76%。非B治疗组的pEFS为60%(p = 0.22)。Ⅰ期和Ⅱ期儿童的预后明显更好。Ⅲ期和Ⅳ期之间无统计学差异。除大细胞淋巴瘤外,NHL各亚型的治疗结果具有可比性,大细胞淋巴瘤的预后明显更好(pEFS为90%)。

结论

在捷克共和国使用基于BFM 90研究的方案是可行的。pEFS比德国研究低约10%,但与其他一些研究相当。大细胞淋巴瘤的预后极佳。需要减少治疗相关并发症和死亡率,并进行更精确的分类。

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