The biology of pediatric acute megakaryoblastic leukemia

被引:102
作者
Gruber, Tanja A. [1 ,2 ]
Downing, James R. [2 ]
机构
[1] St Jude Childrens Res Hosp, Dept Oncol, Memphis, TN 38105 USA
[2] St Jude Childrens Res Hosp, Dept Pathol, Memphis, TN 38105 USA
关键词
ACUTE MYELOID-LEUKEMIA; TRANSIENT MYELOPROLIFERATIVE DISORDER; ACUTE MEGAKARYOCYTIC LEUKEMIA; X-LINKED THROMBOCYTOPENIA; SERUM RESPONSE FACTOR; ETS-RELATED GENE; DOWN-SYNDROME; TRANSCRIPTION FACTOR; GATA1; MUTATIONS; COHESIN COMPLEX;
D O I
10.1182/blood-2015-05-567859
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Acute megakaryoblastic leukemia (AMKL) comprises between 4% and 15% of newly diagnosed pediatric acute myeloid leukemia patients. AMKL in children with Down syndrome (DS) is characterized by a founding GATA1 mutation that cooperates with trisomy 21, followed by the acquisition of additional somatic mutations. In contrast, non-DS-AMKL is characterized by chimeric oncogenes consisting of genes known to play a role in normal hematopoiesis. CBFA2T3-GLIS2 is the most frequent chimeric oncogene identified to date in this subset of patients and confers a poor prognosis.
引用
收藏
页码:943 / 949
页数:7
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