Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.
Department of Pediatrics, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
Cancer. 2022 Mar 15;128(6):1294-1301. doi: 10.1002/cncr.34042. Epub 2021 Nov 30.
Children with leukemia and Down syndrome (DS) are at higher risk of acute treatment toxicities than those without DS. Whether late toxicity risks are also elevated is unknown.
The authors identified all patients diagnosed with leukemia before the age of 18 years in Ontario, Canada between 1987 and 2013 and who survived greater than 5 years since their last pediatric cancer event. Survivors were divided into those with and without DS, matched by birth year, sex, leukemia type, and receipt of radiation. DS survivors were matched to individuals with DS without childhood cancer (DS controls) in a 1:10 ratio. Outcomes were identified through linkage to population-based health services databases.
DS survivors (n = 79) experienced inferior overall survival compared to non-DS survivors (n = 231) (20-year overall survival, 81.7% ± 6.8% vs 98.3% ± 1.2%; hazard ratio [HR], 12.8; P < .0001) and to DS controls (n = 790; 96.3% ± 1.2%; HR, 5.4 P < .0001). Pulmonary and infectious deaths were noted among DS survivors. There was no difference in the incidence of congestive heart failure between DS survivors and either control cohort, nor of hearing loss or dementia between DS survivors and DS controls.
DS survivors were at substantially higher risk of late mortality than non-DS survivors or DS controls. This excess risk was not attributable to cardiac- or subsequent malignant neoplasm-related late effects, historically main causes of premature death among non-DS survivors. Chronic morbidities associated with DS were not increased compared to DS controls. DS-specific surveillance guidelines may be warranted.
患有白血病和唐氏综合征（DS）的儿童比没有 DS 的儿童更容易发生急性治疗毒性。但目前尚不清楚其晚期毒性风险是否也会升高。
作者在加拿大安大略省，从 1987 年到 2013 年期间，确定了所有 18 岁以下被诊断患有白血病并在最后一次儿科癌症事件后存活 5 年以上的患者。将幸存者分为有无 DS 两组，通过出生年份、性别、白血病类型和是否接受放疗进行匹配。将 DS 幸存者与没有儿童癌症的 DS 个体（DS 对照组）按 1:10 的比例进行匹配。通过与基于人群的健康服务数据库的链接来确定结局。
DS 幸存者（n = 79）的总生存率明显低于非 DS 幸存者（n = 231）（20 年总生存率，81.7% ± 6.8% vs 98.3% ± 1.2%；风险比[HR]，12.8；P <.0001）和 DS 对照组（n = 790）（96.3% ± 1.2%；HR，5.4；P <.0001）。DS 幸存者中发生了肺部和感染性死亡。DS 幸存者充血性心力衰竭的发生率与任何对照组之间无差异，DS 幸存者与 DS 对照组之间听力损失或痴呆的发生率也无差异。
DS 幸存者的晚期死亡率明显高于非 DS 幸存者或 DS 对照组。这种超额风险不能归因于心脏或随后的恶性肿瘤相关的晚期效应，这是导致非 DS 幸存者过早死亡的主要原因。与 DS 对照组相比，DS 幸存者的慢性合并症并未增加。可能需要制定针对 DS 的特定监测指南。
