Prognostic Impact of Intensified Maintenance Therapy on Children With Advanced Lymphoblastic Lymphoma: A Report From the Japanese Pediatric Leukemia/Lymphoma Study Group ALB-NHL03 Study.
Prognostic Impact of Intensified Maintenance Therapy on Children With Advanced Lymphoblastic Lymphoma: A Report From the Japanese Pediatric Leukemia/Lymphoma Study Group ALB-NHL03 Study.
复制标题
强化维持治疗对晚期淋巴母细胞淋巴瘤儿童的预后影响：来自日本小儿白血病/淋巴瘤研究组 ALB-NHL03 研究的报告。
DOI：
10.1002/pbc.25824
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发表时间：
2016
影响因子：
3.2
通讯作者：
Tsurusawa M
中科院分区：
文献类型：
--
作者：
Sunami S;Sekimizu M;Takimoto T;Mori T;Mitsui T;Fukano R;Saito AM;Watanabe T;Ohshima K;Fujimoto J;Nakazawa A;Kobayashi R;Horibe K;Tsurusawa M
儿童晚期淋巴母细胞性淋巴瘤（LBL）在急性淋巴母细胞性白血病（ALL）的治疗策略中具有良好的预后，无事件生存率（EFS）超过80%。然而，在过去的10 years.ProcedureWe进行了第一个全国性的前瞻性研究儿童晚期LBL的疗效和安全性评估ALL导向治疗与强化维持阶段。除了患有初始中枢神经系统疾病的患者外，我们省略了局部放疗，包括预防性头颅放疗。治疗的总持续时间为24 months.ResultsFor在这项研究中分析的136例患者，5年总生存率（OS）为82.9%，5年EFS为77.9%。观察到30起事件，其中14起发生在强化维持阶段开始前。在14例事件中，观察到9例纵隔扩大。当根据性别或免疫表型分层时，结果无显著差异。根据临床分期，T细胞LBL（T-LBL）患者的5年EFS为III期70.6%，IV期88.9%（P= 0.037）。然而，我们的强化维持治疗不能改善生存结局。日本T-LBL IV期患者的EFS有优于T-LBL III期患者的趋势。
BackgroundChildhood advanced lymphoblastic lymphoma (LBL) has a favorable outcome with an event‐free survival (EFS) rate of over 80% in response to treatment strategies for acute lymphoblastic leukemia (ALL). However, no progress has been made in this outcome over the past 10 years.ProcedureWe conducted the first nationwide prospective study of childhood advanced LBL to assess the efficacy and safety of ALL‐directed therapy with an intensified maintenance phase. We omitted local radiotherapy including prophylactic cranial radiotherapy except for patients with initial central nervous system disease. The total duration of the treatment was 24 months.ResultsFor the 136 patients analyzed in this study, 5‐year overall survival (OS) was 82.9% and 5‐year EFS was 77.9%. Thirty events were observed and 14 occurred before the initiation of intensified maintenance phase. Of 14 events, nine were observed as mediastinal enlargement. There was no significant difference in outcome when stratified according to gender or by immunophenotype. The 5‐year EFS according to clinical stage in patients with T‐cell LBL (T‐LBL) was 70.6% for stage III and 88.9% for stage IV (P= 0.037).ConclusionsOur first nationwide study provided about 80% cure rate with only one case of toxic death in childhood advanced LBL. However, our intensified maintenance therapy could not improve the survival outcome. There was a trend of better EFS in Japanese patients with T‐LBL stage IV than T‐LBL stage III.