title: WT1 gene expression in children with Down syndrome and transient myeloproliferative disorder.
abstract: Transient myeloproliferative disorder (TMD) is found in 10% of newborns with Down syndrome (DS). Myeloid leukemia develops in 25% within the following 3 years. Little is known about markers predicting leukemia occurrence. We studied expression levels of the Wilms tumor gene (WT1) by real-time quantitative PCR (RQ-PCR) in peripheral blood of five infants with TMD. WT1 levels were elevated similar to findings in AML. Longitudinal studies showed normalization of the WT1 level in all patients except one who developed GATA1 mutated myeloid leukemia at 11 months of age. The lack of normalization of WT1 level may be a predictor of leukemia development and WT1 expression may be an attractive marker for monitoring of minimal residual disease.
authors.affiliation: Department of Pediatrics, Skejby Hospital, DK-8200 Aarhus N, Denmark.
authors.forename: Henrik
authors.initials: H
authors.lastname: Hasle
authors.forename: Bendik
authors.initials: B
authors.lastname: Lund
authors.forename: Charlotte Guldborg
authors.initials: CG
authors.lastname: Nyvold
authors.forename: Peter
authors.initials: P
authors.lastname: Hokland
authors.forename: Mette
authors.initials: M
authors.lastname: Østergaard
mesh_terms: D000208:Acute Disease; D003561:Cytarabine / Q000627:therapeutic use; D004314:Down Syndrome / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D050982:GATA1 Transcription Factor / Q000235:genetics; D020869:Gene Expression Profiling; D015973:Gene Expression Regulation, Leukemic / Q000235:genetics*; D016162:Genes, Wilms Tumor; D006801:Humans; D007231:Infant, Newborn; D007951:Leukemia, Myeloid / Q000097:blood / Q000175:diagnosis / Q000235:genetics*; D008137:Longitudinal Studies; D008297:Male; D009154:Mutation; D009196:Myeloproliferative Disorders / Q000097:blood / Q000175:diagnosis / Q000235:genetics*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D016896:Treatment Outcome
chemical_list: D050982:GATA1 Transcription Factor; D003561:Cytarabine