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儿童和青少年非霍奇金淋巴瘤:根据生物学亚型和分期分层治疗的结果——柏林-法兰克福-明斯特集团报告

Non-Hodgkin's lymphomas of childhood and adolescence: results of a treatment stratified for biologic subtypes and stage--a report of the Berlin-Frankfurt-Münster Group.

作者信息

Reiter A, Schrappe M, Parwaresch R, Henze G, Müller-Weihrich S, Sauter S, Sykora K W, Ludwig W D, Gadner H, Riehm H

机构信息

Department of Pediatric Hematology and Oncology, Medizinische Hochschule, Hannover, Germany.

出版信息

J Clin Oncol. 1995 Feb;13(2):359-72. doi: 10.1200/JCO.1995.13.2.359.

DOI:10.1200/JCO.1995.13.2.359
PMID:7844597
Abstract

PURPOSE

To prove the efficacy of a treatment stratified according to histology for children with non-Hodgkin's lymphoma (NHL), including acute B-cell leukemia (B-ALL).

PATIENTS AND METHODS

From October 1986 to March 1990, 302 assessable patients, 0.6 to 17.8 years of age, with newly diagnosed NHL were enrolled onto study ALL/NHL-BFM 86. Fifty percent of patients had Burkitt-type lymphomas, including B-ALL; 24% had lymphoblastic lymphoma; 18% had diffuse large-cell lymphoma; and 8% had an NHL not further classified. Therapy group B included Burkitt's-type lymphomas, B-ALL, and most large-cell lymphomas including Ki-1 anaplastic large-cell lymphoma. Patients with stage I and II disease resected received three, while all others received six, 5-day therapy courses (dexamethasone, methotrexate [MTX] 0.5 g/m2 [5 g/m2 for stage IV and B-ALL], and intrathecal [IT] therapy in each course, plus ifosfamide, cytarabine, and etoposide alternating with cyclophosphamide and doxorubicin). Therapy for group non-B patients (lymphoblastic lymphoma and pleomorphic T-cell lymphoma [PTCL]) consisted of a Berlin-Frankfurt-Münster (BFM) acute lymphoblastic leukemia protocol, including cranial irradiation for advanced stage. Local therapy was restricted to patients with incomplete tumor regression.

RESULTS

The probabilities of event-free survival (pEFS) at 7 years were 80% +/- 2% for the whole group, 81% +/- 3% for group B (n = 225), and 78% +/- 5% for group non-B (n = 77) with a follow-up duration of 3.6 to 7 years (median 5 years). Treatment results were comparable between NHL subtypes, except for PTCL, in which three of four patients suffered from relapse. Local disease manifestations were the most frequent site of failure.

CONCLUSION

This therapy strategy provided patients of all NHL subtypes with an equally high chance to survive event-free, except patients with PTCL. With reduced systemic failure, local tumor control may become more important.

摘要

目的

证明根据组织学对非霍奇金淋巴瘤(NHL)患儿(包括急性B淋巴细胞白血病(B-ALL))进行分层治疗的疗效。

患者与方法

1986年10月至1990年3月,302例年龄在0.6至17.8岁之间、新诊断为NHL的可评估患者被纳入ALL/NHL-BFM 86研究。50%的患者患有伯基特型淋巴瘤,包括B-ALL;24%患有淋巴母细胞淋巴瘤;18%患有弥漫性大细胞淋巴瘤;8%患有未进一步分类的NHL。治疗组B包括伯基特型淋巴瘤、B-ALL以及大多数大细胞淋巴瘤(包括Ki-1间变性大细胞淋巴瘤)。I期和II期疾病已切除的患者接受3个疗程,而其他所有患者接受6个疗程的5天治疗(地塞米松、甲氨蝶呤[MTX]0.5 g/m²[IV期和B-ALL为5 g/m²],每个疗程进行鞘内[IT]治疗,加用异环磷酰胺、阿糖胞苷和依托泊苷,并与环磷酰胺和多柔比星交替使用)。非B组患者(淋巴母细胞淋巴瘤和多形性T细胞淋巴瘤[PTCL])接受柏林-法兰克福-明斯特(BFM)急性淋巴细胞白血病方案治疗,包括对晚期患者进行颅脑照射。局部治疗仅限于肿瘤未完全消退的患者。

结果

全组7年无事件生存率(pEFS)为80%±2%,B组(n = 225)为81%±3%;非B组(n = 77)为78%±5%,随访时间为3.6至7年(中位时间5年)。除PTCL外(4例患者中有3例复发),NHL各亚型的治疗结果具有可比性。局部疾病表现是最常见的失败部位。

结论

除PTCL患者外,该治疗策略为所有NHL亚型患者提供了同样高的无事件生存机会。随着全身失败率的降低,局部肿瘤控制可能变得更加重要。

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