Department of Pediatric Oncology/Hematology, Erasmus MC/Sophia Children's Hospital, Dr Molewaterplein 60, Rotterdam, The Netherlands.
Hematol Oncol Clin North Am. 2010 Feb;24(1):19-34. doi: 10.1016/j.hoc.2009.11.009.
Children with Down syndrome have an increased risk for developing both acute myeloid as well as lymphoblastic leukemia. These leukemias differ in presenting characteristics and underlying biology when compared with leukemias occurring in non-Down syndrome children. Myeloid leukemia in children with Down syndrome is preceded by a preleukemic clone (transient leukemia or transient myeloproliferative disorder), which may disappear spontaneously, but may also need treatment in case of severe symptoms. Twenty percent of children with transient leukemia subsequently develop myeloid leukemia. This transition offers a unique model to study the stepwise development of leukemia and of gene dosage effects mediated by aneuploidy.
唐氏综合征患儿发生急性髓系白血病和急性淋巴细胞白血病的风险增加。与非唐氏综合征患儿发生的白血病相比，这些白血病在临床表现和基础生物学方面存在差异。唐氏综合征患儿的髓系白血病之前存在白血病前克隆（短暂性白血病或短暂性骨髓增生异常），可能会自行消失，但如果出现严重症状，也可能需要治疗。20%的短暂性白血病患儿随后会发展为髓系白血病。这种转变为研究白血病的逐步发展以及非整倍体介导的基因剂量效应提供了一个独特的模型。
