唐氏综合征合并急性白血病的预后
Prognosis of Down's syndrome with acute leukaemia.
作者信息
Levitt G A, Stiller C A, Chessells J M
机构信息
Department of Haematology and Oncology, Hospital for Sick Children, London.
出版信息
Arch Dis Child. 1990 Feb;65(2):212-6. doi: 10.1136/adc.65.2.212.
The outcome in children with acute leukaemia with (n = 90) and without Down's syndrome (n = 4377) was compared. Sixty three (70%) of those with Down's syndrome had acute lymphoblastic leukaemia and in comparison with 3664 (84%) controls had similar prognostic features except for a significant excess of the 'common' immunological subtype of acute lymphoblastic leukaemia. The outcome of the children with Down's syndrome was significantly worse with a five year overall actuarial survival of 28% compared with 59% in the control group. It appeared that both suboptimal chemotherapy and a high rate of infective problems contributed to the poor survival. Twenty six children with Down's syndrome had acute myeloblastic leukaemia and were significantly younger and had a higher percentage of the megakaryocytic and erythroid subtypes of acute myeloblastic leukaemia than the 713 controls. The outcome was similar in the two groups. It is concluded that the patients with Down's syndrome who develop acute leukaemia should receive standard protocols without modification, but aggressive supportive care is necessary to improve outcome.
对患有急性白血病的唐氏综合征患儿（n = 90）和非唐氏综合征患儿（n = 4377）的治疗结果进行了比较。63名（70%）唐氏综合征患儿患有急性淋巴细胞白血病，与3664名（84%）对照患儿相比，除急性淋巴细胞白血病的“常见”免疫亚型明显增多外，预后特征相似。唐氏综合征患儿的治疗结果明显更差，五年总精算生存率为28%，而对照组为59%。似乎化疗不充分和感染问题发生率高都导致了生存率低下。26名唐氏综合征患儿患有急性髓细胞白血病，与713名对照患儿相比，年龄明显更小，急性髓细胞白血病的巨核细胞和红系亚型比例更高。两组的治疗结果相似。结论是，患急性白血病的唐氏综合征患者应接受标准方案且无需调整，但需要积极的支持性治疗以改善治疗结果。
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