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在单一机构使用淋巴瘤恶性肿瘤B方案治疗的伯基特淋巴瘤和L3急性淋巴细胞白血病儿童的治疗结果。

Treatment outcomes in children with Burkitt lymphoma and L3 acute lymphoblastic leukemia treated using the lymphoma malignancy B protocol at a single institution.

作者信息

Park Eun Sil, Kim Hyery, Lee Ji Won, Lim Jae-Young, Kang Hyoung Jin, Park Kyung Duk, Shin Hee Young, Ahn Hyo Seop

机构信息

Department of Pediatrics, Institute of Health Science, Gyeongsang National University School of Medicine, Jinju, Korea.

出版信息

Korean J Hematol. 2011 Jun;46(2):96-102. doi: 10.5045/kjh.2011.46.2.96. Epub 2011 Jun 21.

Abstract

BACKGROUND

We compared the outcomes of patients with Burkitt lymphoma and French-American-British (FAB) L3 acute lymphoblastic leukemia treated using Lymphoma Malignancy B (LMB) or other treatment protocols.

METHODS

Thirty-eight patients diagnosed between July 1996 and December 2007 were treated using LMB 96, and 22 patients diagnosed between January 1991 and May 1998 (defined as the early period) were treated using the D-COMP or CCG-106B protocols. We retrospectively reviewed their medical records and analyzed cumulative survival according to the treatment period by using Kaplan-Meier analysis.

RESULTS

There were no intergroup differences in the distribution of age, disease stage, or risk group. The median follow-up period of the 33 live patients in the LMB group was 72 months (range, 36-170 months). Overall survival (OS) and event-free survival (EFS) of patients treated using LMB 96 were 86.8%±5.5% and 81.6%±6.3%, respectively, whereas OS and EFS of patients treated in the early period were 72.7%±9.6% and 68.2%±9.9%, respectively. In the LMB 96 group, OS of cases showing non-complete response (N=8) was 62.5%±17.1%, and OS of relapsed or primary refractory cases (N=6) was 33.3%±19.3%. Central nervous system (CNS) disease, high lactate dehydrogenase levels at diagnosis, and treatment response were significant prognostic factors.

CONCLUSION

Survival outcome has drastically improved over the last 2 decades with short-term, dose-intensive chemotherapy. However, CNS involvement or poor response to chemotherapy was worse prognostic factors; therefore, future studies addressing this therapeutic challenge are warranted.

摘要

背景

我们比较了采用淋巴瘤恶性肿瘤B(LMB)方案或其他治疗方案治疗的伯基特淋巴瘤患者以及法国-美国-英国(FAB)L3急性淋巴细胞白血病患者的治疗结果。

方法

1996年7月至2007年12月期间诊断的38例患者采用LMB 96方案治疗,1991年1月至1998年5月期间(定义为早期)诊断的22例患者采用D-COMP或CCG-106B方案治疗。我们回顾性审查了他们的病历,并采用Kaplan-Meier分析方法根据治疗时期分析累积生存率。

结果

年龄、疾病分期或风险组的分布在组间无差异。LMB组33例存活患者的中位随访期为72个月(范围36 - 170个月)。采用LMB 96方案治疗的患者的总生存期(OS)和无事件生存期(EFS)分别为86.8%±5.5%和81.6%±6.3%,而早期治疗患者的OS和EFS分别为72.7%±9.6%和68.2%±9.9%。在LMB 96组中,未达到完全缓解的病例(N = 8)的OS为62.5%±17.1%,复发或原发性难治性病例(N = 6)的OS为33.3%±19.3%。中枢神经系统(CNS)疾病、诊断时乳酸脱氢酶水平高以及治疗反应是显著的预后因素。

结论

在过去20年中,短期、剂量密集化疗使生存结果有了显著改善。然而,CNS受累或化疗反应不佳是较差的预后因素;因此,有必要开展针对这一治疗挑战的未来研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/731b/3128907/a117d0d1bfcf/kjh-46-96-g001.jpg

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