[Expression and Significance of Shh Signaling Pathway in Bone Marrow CD34 Cells of Patients with Myelodysplastic Syndrome and Acute Myeloid Leukemia with Myelodysplasia-Related Changes].

Objective: To study the expression of Shh singaling related gene, including Shh, Ptch1, Smo and Gli1 in bone marrow CD34 cells of patients with myelodysplastic syndrome(MDS) and acute myeloid leukemia with myelodysplasia-related changes(AML-MRC), and to explore their clinical significance.

Methods: The count of CD34 cells in bone marrow was detected by flow cytometry in 53 patients with MDS and 30 patients with AML-MRC. Magnetic beads were used to separate CD34 cells. The expression of Shh, Ptch1,Smo and Gli1 on CD34 cells was detected by qRT-PCR, the effect of the 4 gene expression on prognosis of patients in MDS and AML-MRC group was compared, 25 patients with iron-deficiency anemia were used as controls.

Results: The expression levels of Shh, Smo and Gli1 of patients in MDS and AML-MRC group were significantly higher than those in control group (P＜0.05), moreover increased with disease progression(P<0.05). The expression of Ptch1 was not statistically significantly different between 3 groups（P＞0.05）. In comparison of prognosis, the expression of Smo and Gli1 in the patients of relatively high risk groups and AML-MRC groups were significantly increased (P<0.05). The median overall survival time of patients in MDS and AML-MRC groups was 12（7.5，16.5） and 6（3.0，9.0） months (P=0.000) respectively. The median survival time of MDS and AML-MRC patients with high expression of Smo and Gli1 was significantly shorter than that of MDS and AML-MRC patients with low expression of Smo and Gli1(P<0.05).

Conclusion: Shh signaling pathway in the patients with MDS is activated, which is involved in the progress and prognosis of MDS.