ABSTRACT

Childhood lymphoblastic lymphoma (LL) is a highly aggressive neoplasm which has achieved favorable survival outcomes in many developed countries. However, few studies have reported treatment outcomes of childhood LL in resource-limited counties, nor has a prognostic scoring system been developed. The objectives of this study were to evaluate survival outcomes and identify prognostic factors associated with inferior outcomes of childhood LL in a referral center in March 1985 and April 2017 were retrospectively reviewed. Seventy-five advanced-stage LL patients were included, 47 (62.7%) of whom had stage IV at initial diagnosis. The 5-year DFS and OS rates were 44.6% and 44.7%, respectively. There were 3 significant prognostic factors associated with worse outcomes: presence of B symptoms, low albumin level < 3.5 g/dL and serum LDH level > 500 IU/L. From these three factors, we assigned a score of 1 for each and total scores of 0, 1, 2, and 3 could predict 5-year OS rates of 92.3%, 50.9%, 24.7% and 0%, respectively (p < 0.05). The survival of children in this study was lower than in other studies of advanced-stage childhood LL. We identified 3 adverse prognostic factors and developed a prognostic model for clinical use in advanced-stage childhood LL.

摘要

儿童淋巴母细胞淋巴瘤 (LL) 是一种高度侵袭性的肿瘤，在许多发达国家都取得了良好的生存结果。然而，很少有研究报告资源有限县儿童 LL 的治疗结果，也没有开发预后评分系统。本研究的目的是评估生存结果并确定与 1985 年 3 月和 2017 年 4 月转诊中心儿童 LL 不良结果相关的预后因素。包括 75 名晚期 LL 患者，其中 47 名（62.7%）在初始诊断时为 IV 期。5 年 DFS 和 OS 率分别为 44.6% 和 44.7%。有 3 个显着的预后因素与较差的结果相关：存在 B 症状、低白蛋白水平 < 3.5 g/dL 和血清 LDH 水平 > 500 国际单位/升。从这三个因素中，我们为每个因素分配了 1 分，总分 0、1、2 和 3 可以预测 5 年 OS 率分别为 92.3%、50.9%、24.7% 和 0%（p <  0.05)。本研究中儿童的存活率低于其他晚期儿童 LL 研究。我们确定了 3 个不良预后因素，并开发了一个用于晚期儿童 LL 临床使用的预后模型。