title: [Clinical significance of the Wilms' tumor 1 mRNA expression in childhood myelodysplastic syndrome].
abstract: OBJECTIVE
  To investigate the expression of the Wilms' tumor 1 (WT1) mRNA in childhood myelodysplastic syndrome (MDS), and to evaluate WT1 as a tool to differentiate MDS from aplastic anemia(AA).
  
  
  METHODS
  The quantitative expression of WT1 transcript by using real-time quantitative polymerase chain reaction (RQ-PCR) was performed in the bone marrow samples of 36 childhood MDS and 49 childhood AA, the samples were collected from September 2008 to December 2011.
  
  
  RESULTS
  (1) The positive rate of WT1 in severe AA (SAA) was 0, 14.3% in chronic AA (CAA), 58.6% in refractory cytopenia (RC), 100% in refractory anemia with excessive blast (RAEB) and 97.5% in acute myeloid leukemia (AML). The mean level of WT1 in SAA, CAA, RC, RAEB and AML was 0.041%, 0.357%, 7.037%, 12.680% and 24.210%, respectively. The positive rate of WT1 in RC patients was higher than that of SAA (P = 0.000) and CAA (P = 0.001). (2) The positive rate of WT1 in patients with hypoplastic MDS was 66.7% and was higher than that of SAA (P = 0.000) and CAA (P = 0.001). The mean level of WT1 in patients with hypoplastic MDS was (3.022 ± 5.040)% and higher than that of SAA \[(0.041 ± 0.047)%, P = 0.000\] and CAA\[(0.351 ± 0.479)%, P = 0.002\].
  
  
  CONCLUSIONS
  The level of WT1 in childhood MDS was higher than that of childhood AA. The degree of WT1 expression in MDS increased during disease progression. WT1 is a useful tool for differentiating the childhood hypoplastic MDS from AA.
authors.affiliation: Department of Pediatrics, The Affiliated People's Hospital of Peking University, Beijing 100044, China.
authors.forename: Yi-fei
authors.initials: YF
authors.lastname: Cheng
authors.forename: Le-ping
authors.initials: LP
authors.lastname: Zhang
authors.forename: Yan-rong
authors.initials: YR
authors.lastname: Liu
authors.forename: Ya-zhen
authors.initials: YZ
authors.lastname: Qin
authors.forename: Jun
authors.initials: J
authors.lastname: Wu
authors.forename: Gui-lan
authors.initials: GL
authors.lastname: Liu
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009190:Myelodysplastic Syndromes / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D025721:WT1 Proteins / Q000235:genetics / Q000378:metabolism*
chemical_list: D025721:WT1 Proteins