血液病骨髓间质超微病理——附13例病例分析

Ultrastructural pathology of bone marrow stroma in patient with hematological disease——a study of 13 cases

目的:研究血液病患者骨髓间质超微病理特点。方法:透射电子显微镜观察13例血液病患者骨髓活检组织。结果:所有患者髓窦、造血岛和血管关系紊乱,成纤维细胞增多;部分患者出现成纤维细胞活化和肌成纤维细胞转化,与胶原积聚和不定形细胞外基质增多相关。白血病患者骨髓同时存在白血病细胞增生和破坏;骨髓增生异常综合征粒、红细胞凋亡显著,嗜酸粒细胞增多;真性红细胞增多症骨髓晚幼红裸核增多,巨噬细胞吞噬活跃;原发性骨髓纤维化骨髓嗜酸粒细胞和巨核细胞释放大量致密颗粒。结论:血液病患者骨髓结构紊乱,成纤维细胞不同程度增生,成纤维细胞活化和肌成纤维细胞转化与细胞外基质积聚相关;纤维细胞的多克隆增生和纤维化可能继发于大量血细胞破坏和活性物质释放引起的炎性反应。

Objective：To investigate the ultrastructural characteristics of bone marrow （BM） stroma in varied hematological disease. Method： Observing BM biopsy from 13 patients with different hematological disease by transmission electron microscope. Result： The structural relationship between blood sinus, islets and capillary in BM was deformed and fibroblasts were increased in all cases. Some cases exhibited activated fibroblasts and myofibroblasts transformation, which were consistent with the accumulation of collagen and extracellular amorphous matrix in BM. Hyperplasia and destruction of blood cell were coexistent in leukemia cases. Granulocytes and erythrocytes apoptosis and eosinophil were predominant in myelodisplasia syndrome. Naked nucleus from late erythroblast and phagocytes with late erythrocytes or red cells were notable in polycythemia vera. The degranulation of megakaryocytes or eosinophil was prevalent in BM of patients with idiopathic myelofibrosis. Conclusion：The BM structure was disorder and fibroblasts were increased. Fibroblast activation and myofibroblast transformation were related with accumulation of extracellular matrix in BM of patients with hematological diseases. The polyclonal fibroblast proliferation and meofibrosis maybe resulted from the inflammation caused by excessive cell death and of active material release in BM of patients with hematological disease.