Publication Date:
2005-11-16
Description:
Children with Down Syndrome (DS) are at an 150 fold increased risk to develop acute megakaryoblastic leukemia (AMKL) within the first 4 years of life. About 10% of newborns with trisomy 21 showed transient myeloproliferative disorder (TMD). Although mutations of the transcriptional factor GATA1, resulting in the shortened GATA1s have been shown in almost all blasts in DS-AMKL and TMD the predisposition to leukemiogenesis related to trisomy 21 is not clear. TMD occurred during embryonic stress hematopoiesis leading to the hepatic proliferation of the GATA1s positive blasts. Typically blasts disappeared within the first 3 month of live, however after a median time of 1.3years (0.6 to 3.7 years) 20% of the children suffered AMKL and required intensive cytostatic treatment. The expression of chromosome 21 encoded hematological transcription factors (TFs) such RUNX1, ETS-2 and ERG were analysed in leukemic blasts from DS- TMD(n=7), DS-AMKL (n=25), DS without hematological disorder (n=10), AMKL (n=10) and healthy controls (n=7) by qRT-PCR. Results: No increase of RUNX1, ETS-2 and ERG expression could be shown. By contrast, ERG was decrease in all leukemias and in DS without hematological disorder (p Anova.
Print ISSN:
0006-4971
Electronic ISSN:
1528-0020
Topics:
Biology
,
Medicine
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