Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark.
Department of Women's and Children's Health, Childhood Cancer Research Unit, Karolinska University Hospital and Karolinska Institute, Stockholm, Sweden.
Pediatr Blood Cancer. 2018 Sep;65(9):e27249. doi: 10.1002/pbc.27249. Epub 2018 May 24.
Late health consequences of treatment for childhood leukemia are well documented. Although individuals with Down syndrome (DS) have a substantially increased risk of leukemia, information on late effects in this group is almost nonexistent. The aim of this study was to evaluate the mortality and morbidity among 5-year leukemia survivors with DS.
We compared 5-year leukemia survivors with leukemia-free individuals with DS. All individuals born with DS in Denmark between 1960 and 2007 and in Sweden between 1973 and 2009 were included. Long-term morbidity was estimated by comparing hospitalization rates between survivors and leukemia-free individuals.
In total, we found 6,705 individuals with DS, 84 of whom were 5-year survivors of leukemia. Survivors had a higher risk of death (hazard ratio [HR] 5.9; 95% confidence interval [CI]: 2.7-13) compared with leukemia-free individuals. All deaths (n = 7) among 5-year leukemia survivors were due to relapse. Survivors had a higher hospitalization rate (HR 4.4; 95% CI: 3.1-6.2). However, most of these hospitalizations were due to relapse. Censoring individuals who either had a relapse or were being treated for a relapse more than 5 years from the initial diagnosis (n = 9) attenuated the association (HR 1.4; 95% CI: 1.0-2.1).
In this study, we found that relapse was the main reason for death and hospitalization among leukemia survivors with DS, and not late effects. These results are reassuring for individuals treated for DS associated with leukemia and their parents.
儿童期白血病治疗的晚期健康后果已有充分记录。尽管唐氏综合征（Down syndrome，DS）患者罹患白血病的风险显著增加，但该群体的晚期效应信息几乎不存在。本研究旨在评估患有 DS 的 5 年白血病幸存者的死亡率和发病率。
我们比较了患有 DS 的 5 年白血病幸存者与无白血病 DS 个体。所有在丹麦（1960 年至 2007 年期间）和瑞典（1973 年至 2009 年期间）出生的 DS 个体均被纳入研究。通过比较幸存者和无白血病个体的住院率，评估长期发病率。
我们共发现 6705 名 DS 个体，其中 84 名是 5 年白血病幸存者。与无白血病个体相比，幸存者的死亡风险更高（风险比 [hazard ratio，HR] 5.9；95%置信区间 [confidence interval，CI]：2.7-13）。所有 5 年白血病幸存者（n=7）的死亡均归因于疾病复发。幸存者的住院率更高（HR 4.4；95% CI：3.1-6.2）。然而，这些住院治疗大多归因于疾病复发。对初始诊断后 5 年以上复发或正在接受复发治疗的个体进行删失（n=9），减弱了该关联（HR 1.4；95% CI：1.0-2.1）。
在本研究中，我们发现复发是 DS 白血病幸存者死亡和住院的主要原因，而非晚期效应。这些结果对于接受治疗的 DS 合并白血病患者及其父母来说是令人安心的。
