Acute megakaryoblastic leukemia in Down syndrome
Acute megakaryoblastic leukemia in Down syndrome
复制标题
唐氏综合症急性巨核细胞白血病
作者：
J. Hitzler
患有唐氏综合征（DS）的儿童患急性白血病的风险增加10至20倍。据估计，10%的新生儿患有DS发展为短暂性骨髓增生性疾病（TMD）或短暂性白血病（TL），这是一种巨核细胞的克隆性积聚，在数月内自发消退。急性巨核细胞白血病（AMKL）的发展约20%的TMD/TL的情况下，4岁。DS中AMKL和TMD/TL的原始细胞都含有GATA 1的体细胞突变，GATA 1是巨核细胞分化的重要转录调节因子。DS巨核细胞白血病的独特表型是白血病转化递增过程的独特生物学模型。儿科血液癌症2007;49：1066-1069。© 2007 Wiley利斯公司
Children with Down syndrome (DS) have a 10‐ to 20‐fold increased risk of developing acute leukemia. An estimated 10% of newborns with DS develop Transient Myeloproliferative Disease (TMD) or Transient Leukemia (TL), a clonal accumulation of megakaryoblasts that resolves spontaneously within months. Acute megakaryoblastic leukemia (AMKL) develops in approximately 20% of cases of TMD/TL by 4 years of age. Both the blasts of AMKL and TMD/TL in DS harbor somatic mutations of GATA1, an essential transcriptional regulator of megakaryocytic differentiation. The distinct phenotypes of megakaryoblastic leukemia in DS are a unique biological model of the incremental process of leukemic transformation. Pediatr Blood Cancer 2007;49:1066–1069. © 2007 Wiley‐Liss, Inc.
影响因子：
20.3
作者：
Mundschau, G;Gurbuxani, S;Crispino, JD
通讯作者：
Crispino, JD
影响因子：
20.3
作者：
Taub, JW;Matherly, LH;Ravindranath, Y
通讯作者：
Ravindranath, Y