Cheng Yi-fei, Zhang Le-ping, Liu Yan-rong, Qin Ya-zhen, Wu Jun, Liu Gui-lan
Department of Pediatrics, The Affiliated People's Hospital of Peking University, Beijing 100044, China.
Zhonghua Xue Ye Xue Za Zhi. 2012 Jul;33(7):536-40.
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).
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.
(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].
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.
探讨肾母细胞瘤1（WT1）mRNA在儿童骨髓增生异常综合征（MDS）中的表达情况，并评估WT1作为鉴别MDS与再生障碍性贫血（AA）的工具的价值。
采用实时定量聚合酶链反应（RQ-PCR）检测2008年9月至2011年12月收集的36例儿童MDS和49例儿童AA骨髓样本中WT1转录本的定量表达。
（1）WT1在重型AA（SAA）中的阳性率为0，慢性AA（CAA）中为14.3%，难治性血细胞减少（RC）中为58.6%，难治性贫血伴原始细胞增多（RAEB）中为100%，急性髓系白血病（AML）中为97.5%。SAA、CAA、RC、RAEB和AML中WT1的平均水平分别为0.041%、0.357%、7.037%、12.680%和24.210%。RC患者中WT1的阳性率高于SAA（P = 0.000）和CAA（P = 0.001）。（2）低增生性MDS患者中WT1的阳性率为66.7%，高于SAA（P = 0.000）和CAA（P = 0.001）。低增生性MDS患者中WT1的平均水平为（3.022 ± 5.040）%，高于SAA[（0.041 ± 0.047）%，P = 0.000]和CAA[（0.351 ± 0.479）%，P = 0.002]。
儿童MDS中WT1水平高于儿童AA。MDS中WT1的表达程度在疾病进展过程中增加。WT1是鉴别儿童低增生性MDS与AA的有用工具。
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