Jin Ling, Zhang Rui, Huang Shuang, Yang Jing, Duan Yan-Long, Zhang Yong-Hong
Hematology Center, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.
Zhonghua Zhong Liu Za Zhi. 2012 Feb;34(2):138-42. doi: 10.3760/cma.j.issn.0253-3766.2012.02.013.
To evaluate the clinical characteristics of childhood lymphoblastic lymphoma (LBL) and therapeutic efficacy of BCH-LBL-2003 regimen modified from BFM-90 protocol. The drug-related toxicities and prognostic factors were explored at the same time.
From Janurary 2003 to December 2009, 112 newly diagnosed LBL patients at the Hematology Center of Beijing Children's Hospital were enrolled in this study. The patients were treated with modified BFM-90-LBL protocol.
At a median follow-up of 29 months (1 to 90 months), the patients were evaluated on day 33 and at the end of induction therapy. The bone marrow complete remission (CR) rates were 96.4% and 100%, respectively. Meanwhile, the complete remission (CR) rates of tumor were 77.7% and 94.5%, and the partial remission (PR) rates were 22.3% and 5.5%, respectively. The overall response rate was 100%. The 3-year overall survival (OS) rate was 89.1% and 5-year OS was 87.0%. The 3-year event-free survival (EFS) was 85.4% and 5-year EFS was 83.3%. Eleven cases relapsed during the treatment (4 BM relapses, 3 CNS recurrences, 3 primary site and 1 lymph node of neck and BM). Eleven patients died (3 died of infection and 8 died of progressive disease after relapse). All patients experienced grade 3-4 hematological toxicity. Univariate analysis indicated that lack of CR at the end of induction therapy, immunophenotype, bulky tumor and course of disease had prognostic significance.
Lymphoblastic lymphoma in childhood and adolescence is highly aggressive. BCH-LBL-2003 protocol is very effective. The treatment-associated side effects were tolerable. Patients who didn't get CR at the end of induction therapy, with T-cell immunophenotype, with bulky disease and the course of disease less than 30 days may have a poor prognosis.
评估儿童淋巴细胞白血病（LBL）的临床特征以及源自BFM - 90方案的BCH - LBL - 2003方案的治疗效果。同时探究药物相关毒性和预后因素。
2003年1月至2009年12月，北京儿童医院血液中心112例新诊断的LBL患者纳入本研究。患者接受改良的BFM - 90 - LBL方案治疗。
中位随访29个月（1至90个月），在第33天和诱导治疗结束时对患者进行评估。骨髓完全缓解（CR）率分别为96.4%和100%。同时，肿瘤完全缓解（CR）率分别为77.7%和94.5%，部分缓解（PR）率分别为22.3%和5.5%。总缓解率为100%。3年总生存率（OS）为89.1%，5年OS为87.0%。3年无事件生存率（EFS）为85.4%，5年EFS为83.3%。11例患者在治疗期间复发（4例骨髓复发，3例中枢神经系统复发，3例原发部位复发，1例颈部淋巴结和骨髓复发）。11例患者死亡（3例死于感染，8例死于复发后疾病进展）。所有患者均经历3 - 4级血液学毒性。单因素分析表明，诱导治疗结束时未达到CR、免疫表型、巨大肿瘤和病程具有预后意义。
儿童和青少年淋巴细胞白血病具有高度侵袭性。BCH - LBL - 2003方案非常有效。治疗相关副作用可耐受。诱导治疗结束时未达到CR、具有T细胞免疫表型、患有巨大疾病且病程少于30天的患者预后可能较差。
