Department of Oncology, St Jude Children’s Research Hospital, TN 38105-3678, USA.
Ann Oncol. 2013 Sep;24(9):2425-9. doi: 10.1093/annonc/mdt221. Epub 2013 Jun 20.
Reliable prognostic factors have not been established for advanced-stage pediatric lymphoblastic lymphoma (LL). We analyzed treatment outcomes and potential risk factors in children and adolescents with advanced-stage LL treated over a 40-year period.
From 1962 through 2002, 146 patients (99 boys and 47 girls) with stage III (n = 111) or stage IV (n = 35) LL were treated at St Jude Children's Research Hospital. The five treatment eras were 1962-1975 (no protocol), 1975-1979 (NHL-75), 1979-1984 (Total 10 High), 1985-1992 (Pediatric Oncology Group protocol), and 1992-2002 (NHL13). Age at diagnosis was <10 years in 65 patients and ≥10 years in 81.
Outcomes improved markedly over successive treatment eras. NHL13 produced the highest 5-year event-free survival (EFS) estimate (82.9% ± 6.1% [SE]) compared with only 20.0% ± 8.0% during the earliest era. Treatment era (P < 0.0001) and age at diagnosis (<10 years versus ≥10 years, P = 0.0153) were independent prognostic factors, whereas disease stage, lactate dehydrogenase level, and presence of a pleural effusion were not.
Treatment era and age were the most important prognostic factors for children with advanced-stage LL. We suggest that a better assessment of early treatment response may help to identify patients with drug-resistant disease who require more intensive therapy.
对于晚期儿科淋巴母细胞淋巴瘤（LL），尚未建立可靠的预后因素。我们分析了 40 年来接受治疗的患有晚期 LL 的儿童和青少年的治疗结果和潜在风险因素。
1962 年至 2002 年，在圣裘德儿童研究医院治疗了 146 例 III 期（n = 111）或 IV 期（n = 35）LL 患儿（99 名男孩和 47 名女孩）。五个治疗时期分别为 1962-1975 年（无方案）、1975-1979 年（NHL-75）、1979-1984 年（全 10 高）、1985-1992 年（儿科肿瘤组方案）和 1992-2002 年（NHL13）。诊断时年龄<10 岁的患者有 65 例，≥10 岁的患者有 81 例。
随着治疗时代的相继发展，结果明显改善。与最早时期的 20.0%±8.0%相比，NHL13 产生了最高的 5 年无事件生存（EFS）估计值（82.9%±6.1%[SE]）。治疗时代（P<0.0001）和诊断时的年龄（<10 岁与≥10 岁，P=0.0153）是独立的预后因素，而疾病分期、乳酸脱氢酶水平和胸腔积液的存在则不是。
治疗时代和年龄是晚期 LL 患儿最重要的预后因素。我们建议，对早期治疗反应的更好评估可能有助于识别需要更强化治疗的耐药性疾病患者。
