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患有唐氏综合征的人群中白血病和固体瘤的风险
H Hasle1, I H Clemmensen, M Mikkelsen
1Department of Paediatrics, Aarhus University Hospital Skejby, Denmark. hasle@post2.tele.dk
Lancet (London, England)
|February 16, 2000
概括
患有唐氏综合征的人患儿白血病的风险明显高,但固体瘤的风险较低. 唐氏综合征中这种独特的癌症模式可能有助于了解影响癌症发展的遗传因素.
科学领域:
- 在瘤学瘤学.
- 遗传学 是一个遗传学.
- 儿科 儿科 儿科
背景情况:
- 与一般人群相比,患有唐氏综合征的人患白血病的风险较高.
- 有限的数据存在于特定年龄的白血病风险和固体瘤风险在这个人口.
研究的目的:
- 估计患有唐氏综合征的个体中各种癌症的特定年龄风险.
- 在这个人群中调查白血病和固体瘤的模式.
主要方法:
- 利用丹麦细胞遗传注册和丹麦癌症注册数据对2814名唐氏综合征患者进行了分析.
- 根据一般人口的发病率计算出标准化发病率 (SIR) 和95%置信区间 (CI).
- 分析了不同年龄组和癌症类型的癌症发病率.
主要成果:
- 总的来说,癌症发病率略有增加 (SIR 1.20),主要原因是白血病风险高,特别是在儿童中 (SIR 56为0-4岁).
- 30岁时白血病的累积风险为2.7%,急性髓性白血病在幼儿中比急性淋巴细胞白血病更频繁.
- 观察到实体瘤风险显著降低 (SIR 0.50),没有发现乳腺癌病例.
结论:
- 唐氏综合征的癌症发生具有独特的特征:儿童白血病风险高,固体瘤风险降低.
- 唐氏综合征中明显的癌症模式可能为在21号染色体上识别白血病和瘤抑制基因提供有价值的线索.
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