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. 1990 Feb;65(2):212–216. doi: 10.1136/adc.65.2.212

Prognosis of Down's syndrome with acute leukaemia.

G A Levitt 1, C A Stiller 1, J M Chessells 1
PMCID: PMC1792207  PMID: 2138446

Abstract

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.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BARBER R., SPIERS P. OXFORD SURVEY OF CHILDHOOD CANCERS; PROGRESS REPORT II. Mon Bull Minist Health Public Health Lab Serv. 1964 Mar;23:46–52. [PubMed] [Google Scholar]
  2. Baird P. A., Sadovnick A. D. Life expectancy in Down syndrome. J Pediatr. 1987 Jun;110(6):849–854. doi: 10.1016/s0022-3476(87)80395-5. [DOI] [PubMed] [Google Scholar]
  3. Blatt J., Albo V., Prin W., Orlando S., Wollman M. Excessive chemotherapy-related myelotoxicity in children with Down syndrome and acute lymphoblastic leukaemia. Lancet. 1986 Oct 18;2(8512):914–914. doi: 10.1016/s0140-6736(86)90429-0. [DOI] [PubMed] [Google Scholar]
  4. Chessells J. M., Leiper A. D. Infection during remission induction in childhood leukaemia. Arch Dis Child. 1980 Feb;55(2):118–123. doi: 10.1136/adc.55.2.118. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Eden O. B., Stiller C. A., Gerrard M. P. Improved survival for childhood acute lymphoblastic leukemia: possible effect of protocol compliance. Pediatr Hematol Oncol. 1988;5(2):83–91. doi: 10.3109/08880018809031258. [DOI] [PubMed] [Google Scholar]
  6. Epstein L. B., Philip R. Abnormalities of the immune response to influenza antigen in Down syndrome (trisomy 21). Prog Clin Biol Res. 1987;246:163–182. [PubMed] [Google Scholar]
  7. KRIVIT W., GOOD R. A. Simultaneous occurrence of mongolism and leukemia; report of a nationwide survey. AMA J Dis Child. 1957 Sep;94(3):289–293. doi: 10.1001/archpedi.1957.04030040075012. [DOI] [PubMed] [Google Scholar]
  8. Levin S. The immune system and susceptibility to infections in Down's syndrome. Prog Clin Biol Res. 1987;246:143–162. [PubMed] [Google Scholar]
  9. Lewis D. S. Association between megakaryoblastic leukaemia and Down syndrome. Lancet. 1981 Sep 26;2(8248):695–695. doi: 10.1016/s0140-6736(81)91027-8. [DOI] [PubMed] [Google Scholar]
  10. Lockitch G., Singh V. K., Puterman M. L., Godolphin W. J., Sheps S., Tingle A. J., Wong F., Quigley G. Age-related changes in humoral and cell-mediated immunity in Down syndrome children living at home. Pediatr Res. 1987 Nov;22(5):536–540. doi: 10.1203/00006450-198711000-00013. [DOI] [PubMed] [Google Scholar]
  11. Miller D. R., Leikin S., Albo V., Sather H., Karon M., Hammond D. Prognostic factors and therapy in acute lymphoblastic leukemia of childhood: CCG-141. A report from childrens cancer study group. Cancer. 1983 Mar 15;51(6):1041–1049. doi: 10.1002/1097-0142(19830315)51:6<1041::aid-cncr2820510612>3.0.co;2-g. [DOI] [PubMed] [Google Scholar]
  12. Montagna D., Maccario R., Ugazio A. G., Nespoli L., Pedroni E., Faggiano P., Burgio G. R. Cell-mediated cytotoxicity in Down syndrome: impairment of allogeneic mixed lymphocyte reaction, NK and NK-like activities. Eur J Pediatr. 1988 Oct;148(1):53–57. doi: 10.1007/BF00441815. [DOI] [PubMed] [Google Scholar]
  13. Ninane J., Chessells J. M. Serious infections during continuing treatment of acute lymphoblastic leukaemia. Arch Dis Child. 1981 Nov;56(11):841–844. doi: 10.1136/adc.56.11.841. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Peeters M., Poon A. Down syndrome and leukemia: unusual clinical aspects and unexpected methotrexate sensitivity. Eur J Pediatr. 1987 Jul;146(4):416–422. doi: 10.1007/BF00444952. [DOI] [PubMed] [Google Scholar]
  15. Peto R., Pike M. C., Armitage P., Breslow N. E., Cox D. R., Howard S. V., Mantel N., McPherson K., Peto J., Smith P. G. Design and analysis of randomized clinical trials requiring prolonged observation of each patient. II. analysis and examples. Br J Cancer. 1977 Jan;35(1):1–39. doi: 10.1038/bjc.1977.1. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Robison L. L., Nesbit M. E., Jr, Sather H. N., Level C., Shahidi N., Kennedy A., Hammond D. Down syndrome and acute leukemia in children: a 10-year retrospective survey from Childrens Cancer Study Group. J Pediatr. 1984 Aug;105(2):235–242. doi: 10.1016/s0022-3476(84)80119-5. [DOI] [PubMed] [Google Scholar]
  17. Rosner F., Lee S. L. Down's syndrome and acute leukemia: myeloblastic or lymphoblastic? Report of forty-three cases and review of the literature. Am J Med. 1972 Aug;53(2):203–218. doi: 10.1016/0002-9343(72)90130-1. [DOI] [PubMed] [Google Scholar]

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