原发性骨髓纤维化和骨髓增生异常综合征伴骨髓纤维化患者纤维驱动细胞的研究

OBJECTIVE: To compare fibrosis-driving cells in patients with primary myelofibrosis（PMF）and patients with myelodysplastic syndromes（MDS）with myelofibrosis（MF）（MDS-MF）. METHODS: Bone marrow biopsy sections of patients with newly diagnosed PMF and MDS（10 each randomly selected for MF-0/1, MF-2, and MF-3）were stained with specific immunofluorescence antibodies to label Gli1, LeptinR, alpha smooth muscle actin（α-SMA）, CD45, and ProcollagenⅠ. Images captured by confocal microscopy were analyzed by Fiji-ImageJ to calculate the cell counts of Gli1(+), LeptinR(+) cells, and fibrosis-driving cells includingα-SMA(+),α-SMA(+)/Gli1(+), α-SMA(+)/LeptinR(+), and ProcollagenⅠ(+)/CD45(+) cells. RESULTS: Patients with PMF and MDS with MF-2/3 had higher LeptinR(+), α-SMA(+), α-SMA(+)/Gli1(+), and ProcollagenⅠ(+)/CD45(+) cell counts compared with those with MF-0/1（all P values<0.05）. However, patients with PMF with MF-2/3 presented with higher Gli1(+) andα-SMA(+)/LeptinR(+) cell counts than those with MF-0/1（P＝0.001 and 0.006）, whereas these cells were similar between patients with MDS with MF-0/1 and MF-2/3（P＝0.169 and 0.067）. In patients with MF-0/1, all fibrosis-driving cells did not differ between PMF and MDS（all P>0.05）. However, in patients with MF-2/3, ProcollagenⅠ(+)/CD45(+) cell counts were higher in patients with PMF compared with those with MDS（P＝0.007）, while other fibrosis-driving cell counts were similar between these two groups（all P>0.05）. MF grade and fibrosis-driving cell counts were not correlated with overall survival in patients with either PMF or MDS. CONCLUSION: α-SMA(+) cells in patients with PMF originated from both Gli1(+) and LeptinR(+) cells, whereasα-SMA(+) cells in patients with MDS-MF only originated from Gli1(+) cells; patients with PMF had higher ProcollagenⅠ(+)/CD45(+) cell counts than those with MDS-MF.