Molecular/cellular biological analysis of pathogenesis and spontaneous remission mechanism in Down syndrome transient myeloproliferative disorder

在患有唐氏综合征的新生儿中，可以看到类似于急性髓性白血病的血液学异常，但通常会自行消失。这种疾病被称为短暂性骨髓增生性疾病（TMD），被认为是一种发生在胎儿肝脏的独特骨髓性肿瘤。其自发缓解的机制尚不清楚。本研究利用细胞培养技术，证实了构成胎肝微环境的基质细胞支持TMD母细胞的生长，而基质细胞产生的造血生长因子之一粒细胞-巨噬细胞集落刺激因子（GM-CSF）在其中发挥了重要作用。这些结果表明，胎儿造血微环境参与了TMD的发生、发展和自发性缓解。

In neonates with Down syndrome, hematological abnormalities similar to acute myeloid leukemia may be seen but usually spontaneously disappear. This disorder, called transient myeloproliferative disorder (TMD), is thought to be a unique myeloid neoplasm arising in the fetal liver. The mechanism of its spontaneous remission is unclear. In this study, using cell culture techniques, we demonstrated that stromal cells constituting microenvironment of the fetal liver support the growth of TMD blasts and that one of the hematopoietic growth factors, granulocyte-macrophage colony-stimulating factor (GM-CSF), produced by the stromal cells play an important role. These results indicate that fetal hematopoietic microenvironment is involved in the onset, progression and spontaneous remission of TMD.