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目的:比较原发性骨髓纤维化（PMF）和骨髓增生异常综合征伴骨髓纤维化（MDS-MF）患者纤维驱动细胞的差异。方法:随机选取MF-0/1、MF-2和MF-3级的初诊PMF和MDS患者各10例，骨髓活检组织切片利用特异性免疫荧光抗体标记Gli1、LeptinR、平滑肌肌动蛋白（α-SMA）、CD45和Procollagen Ⅰ，共聚焦显微镜成像后利用Fiji-ImageJ软件计数Gli1n +、LeptinRn +细胞及α-SMAn +、α-SMAn +/Gli1n +、α-SMAn +/LeptinRn +和ProcollagenⅠn +/CD45n +等纤维驱动细胞。n 结果:MF-2/3级PMF和MDS患者LeptinRn +、α-SMAn +、α-SMAn +/Gli1n +和ProcollagenⅠn +/CD45n +等细胞计数均显著高于MF-0/1级患者（n P0.05）。但MF-2/3级患者中，PMF患者ProcollagenⅠn +/CD45n +计数显著高于MDS患者（n P=0.007），其他纤维驱动细胞计数相比较差异均无统计学意义（n P>0.05）。MF分级及纤维驱动细胞计数与PMF和MDS患者总生存时间无相关性。n 结论:PMF患者α-SMAn +细胞来源于Gli1n +和LeptinRn +细胞，而MDS-MF患者α-SMAn +细胞仅来源于Gli1n +细胞；PMF患者ProcollagenⅠn +/CD45n +细胞计数显著高于MDS-MF患者。n “,”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 Gli1n +, LeptinRn + cells, and fibrosis-driving cells including α-SMA n +, α-SMA n +/Gli1n +, α-SMA n +/LeptinRn +, and ProcollagenⅠn +/CD45n + cells.n Results:Patients with PMF and MDS with MF-2/3 had higher LeptinRn +, α-SMA n +, α-SMA n +/Gli1n +, and Procollagen Ⅰn +/CD45n + cell counts compared with those with MF-0/1 (all n P values0.05) . However, in patients with MF-2/3, Procollagen Ⅰn +/CD45n + cell counts were higher in patients with PMF compared with those with MDS (n P=0.007) , while other fibrosis-driving cell counts were similar between these two groups (all n P>0.05) . MF grade and fibrosis-driving cell counts were not correlated with overall survival in patients with either PMF or MDS.n Conclusion:α-SMAn + cells in patients with PMF originated from both Gli1n + and LeptinRn + cells, whereas α-SMA n + cells in patients with MDS-MF only originated from Gli1n + cells; patients with PMF had higher ProcollagenⅠn +/CD45n + cell counts than those with MDS-MF.n