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[改良BFM-90方案显著改善中国儿童及青少年淋巴细胞性淋巴瘤的治疗效果]

[Modified BFM-90 regimen greatly improves treatment outcomes of chinese childhood and adolescent lymphoblastic lymphoma].

作者信息

Sun Xiao-fei, Zhen Zi-jun, Liu Dong-geng, Xia Zhong-jun, Huang Hui-qiang, Zhang Li, Zhou Zhong-mei, Li Yu-hong, Xia Yi, Ling Jia-yu, Guan Zhong-zhen

机构信息

Department of Medical Oncology , State Key Laboratory of Oncology in Southern China, Cancer Center of Sun Yat-Sen University, Guangzhou 510060, China.

出版信息

Zhonghua Zhong Liu Za Zhi. 2007 Jan;29(1):58-61.

PMID:17575697
Abstract

OBJECTIVE

This study was designed to evaluate the efficacy and toxicity of modified BFM-90 regimen originated from Germany authors in the treatment of Chinese childhood and adolescent lymphoblastic lymphoma.

METHODS

Thirty-six untreated lymphoblastic lymphoma patients aged from 3 to 18 years were included, with 1 patient in stage II , 9 in stage III and 26 in stage IV. Of these 36 patients, 28 (77.7%) were diagnosed as T cell phenotype, 26 (72. 2%) were found to have mediastinal mass, 21 (58. 3%) had bone marrow involvement. All patients received chemotherapy of modified BFM-90 regimen consisting of induction remission, central nerve system prophylaxis, re-induction remission and maintenance therapy. Total treatment duration was two years. The difference from standard BFM-90 is that we omitted cranial radiotherapy but gave regular high dose methotrexate (MTX) iv infusion and intrathecal MTX therapy during maintenance therapy period. Kaplan-Meier method was used to evaluate survival rate.

RESULTS

Of 36 patients, 32 (88%) achieved complete remission (CR) , 1 (2. 7%) partial remission (PR) with an overall response rate of 90.7%. One patient had disease progression ( DP). Two patients received autologous stem cell transplantation at CR1, and two patients received radiotherapy to mediastinum. Totally, 5 patients relapsed, while 2 of them were still alive after salvage chemotherapy. The other 3 died of tumor progression. Two patients died during induction remission, 1 of fungal septicemia, the other of cerebral hemorrhage; one PR and one DP patient died of disease, therefore, totally 7 patients died at last. Median follow-up time was 28 months. Overall three-year survival rate was 78. 3%. The major toxicity was myelosuppression.

CONCLUSION

Modified BFM-90 protocol can improve the efficacy and survival of Chinese childhood and adolescent lymphoblastic lymphoma with tolerable toxicity. However, this modified protocol should only be used in experienced cancer center or hematological unit.

摘要

目的

本研究旨在评估源自德国作者的改良BFM - 90方案治疗中国儿童及青少年淋巴细胞淋巴瘤的疗效和毒性。

方法

纳入36例年龄3至18岁未经治疗的淋巴细胞淋巴瘤患者,其中II期1例,III期9例,IV期26例。这36例患者中,28例(77.7%)诊断为T细胞表型,26例(72.2%)发现有纵隔肿块,21例(58.3%)有骨髓受累。所有患者接受改良BFM - 90方案化疗,包括诱导缓解、中枢神经系统预防、再诱导缓解和维持治疗。总疗程为两年。与标准BFM - 90方案不同的是,我们省略了颅脑放疗,但在维持治疗期间给予常规高剂量甲氨蝶呤(MTX)静脉输注和鞘内MTX治疗。采用Kaplan - Meier法评估生存率。

结果

36例患者中,32例(88%)达到完全缓解(CR),1例(2.7%)部分缓解(PR),总缓解率为90.7%。1例患者疾病进展(DP)。2例患者在CR1时接受自体干细胞移植,2例患者接受纵隔放疗。共有5例患者复发,其中2例在挽救化疗后仍存活。另外3例死于肿瘤进展。2例患者在诱导缓解期死亡,1例死于真菌败血症,另1例死于脑出血;1例PR和1例DP患者死于疾病,因此,最终共有7例患者死亡。中位随访时间为28个月。总体三年生存率为78.3%。主要毒性为骨髓抑制。

结论

改良BFM - 90方案可提高中国儿童及青少年淋巴细胞淋巴瘤的疗效和生存率,且毒性可耐受。然而,这种改良方案仅应在有经验的癌症中心或血液科使用。

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