■小儿间变性大细胞淋巴瘤(ALCL)的风险分层尚未标准化。在这项研究中,纳入新的风险因素,建立新的ALCL风险分层体系,并探讨其在临床实践中的可行性。
■根据非霍奇金淋巴瘤柏林-法兰克福-明斯特95(NHL-BFM-95)协议,轻度播散性疾病(MDD)患者,高危肿瘤部位(多骨,皮肤,肝脏,和肺部受累),和小细胞/淋巴组织细胞(SC/LH)病理亚型纳入危险分层。患者接受改良的NHL-BFM-95方案联合间变性淋巴瘤激酶抑制剂或长春碱(VBL)治疗。
■本研究共纳入136例患者。平均年龄为8.8岁。整个队列的3年无事件生存率(EFS)和总生存率为77.7%[95%置信区间(CI),69.0%-83.9%]和92.3%(95%CI,86.1%-95.8%),分别。低风险组(R1)的3年EFS率,中等风险组(R2),高风险组(R3)患者为100%,89.5%(95%CI,76.5%-95.5%,和67.9%(95%CI,55.4%-77.6%),分别。MDD(+)患者的预后,IV期癌症,SC/LH淋巴瘤,高风险地点很差,3年EFS率为45.3%(95%CI,68.6%-19.0%),65.7%(95%CI,47.6%-78.9%),55.7%(95%CI,26.2%-77.5%),和70.7%(95%CI,48.6%-84.6%),分别。在后续行动结束时,接受VBL维持治疗的5例患者中有1例复发,接受ALK抑制剂维持治疗的7例患者未出现复发.
■这项研究证实了MDD(+)、高危部位和SC/LH的预后不良,但在诊断时患有SC/LH淋巴瘤和MDD(+)的患者仍需要接受更好的治疗(ClinicalTrials.gov编号,NCT03971305)。
UNASSIGNED: The risk stratification of pediatric anaplastic large cell lymphoma (ALCL) has not been standardized. In this study, new risk factors were included to establish a new risk stratification system for ALCL, and its feasibility in clinical practice was explored.
UNASSIGNED: On the basis of the non-Hodgkin\'s lymphoma Berlin-Frankfurt-Munster 95 (NHL-BFM-95) protocol, patients with minimal disseminated disease (MDD), high-risk tumor site (multiple bone, skin, liver, and lung involvement), and small cell/lymphohistiocytic (SC/LH) pathological subtype were enrolled in risk stratification. Patients were treated with a modified NHL-BFM-95 protocol combined with an anaplastic lymphoma kinase inhibitor or vinblastine (VBL).
UNASSIGNED: A total of 136 patients were enrolled in this study. The median age was 8.8 years. The 3-year event-free survival (EFS) and overall survival of the entire cohort were 77.7% [95% Confidence Interval (CI), 69.0%-83.9%] and 92.3% (95% CI,86.1%-95.8%), respectively. The 3-year EFS rates of low-risk group (R1), intermediate-risk group (R2), and high-risk group (R3) patients were 100%, 89.5% (95% CI, 76.5%-95.5%, and 67.9% (95% CI, 55.4%-77.6%), respectively. The prognosis of patients with MDD (+), stage IV cancer, SC/LH lymphoma, and high-risk sites was poor, and the 3-year EFS rates were 45.3% (95% CI, 68.6%-19.0%), 65.7% (95% CI, 47.6%-78.9%), 55.7% (95% CI, 26.2%-77.5%), and 70.7% (95% CI, 48.6%-84.6%), respectively. At the end of follow-up, one of the 5 patients who received maintenance therapy with VBL relapsed, and seven patients receiving ALK inhibitor maintenance therapy did not experience relapse.
UNASSIGNED: This study has confirmed the poor prognostic of MDD (+) ,high risk site and SC/LH ,but patients with SC/LH lymphoma and MDD (+) at diagnosis still need to receive better treatment (ClinicalTrials.gov number, NCT03971305).