骨髓纤维化患者的临床特点和骨髓病理学特征分析

The clinical and pathological features analysis of bone marrow in myelofibrosis

目的探讨骨髓微环境中纤维组织、血管系统的病理改变对部分血液系统疾病的病程及预后的影响。方法重新评估该院诊治的骨髓纤维化患者100例骨髓病理切片,尤其是骨髓微环境中纤维组织、血管系统的病理改变,结合患者的临床症状、肝脾肿大程度、血细胞计数、骨髓细胞学检查及细胞遗传学等相关临床资料,分析骨髓纤维组织增生在血液系统疾病病程网状纤维中的意义。结果所有骨髓活检标本(Gomori)染色均有不同程度阳性,原发性骨髓纤维化(PMF)15例,继发性骨髓纤维化(SMF)85例,SMF病因学分析显示慢性粒细胞白血病、多发性骨髓瘤、原发性血小板增多症易合并骨髓纤维化,SMF纤维化程度较轻,治疗后纤维组织增生程度有所降低。PMF Gomori染色多为强阳性,改善微循环治疗后纤维化程度缓解不显著,差异无统计学意义。结论 PMF较SMF纤维化程度重,SMF与疾病的病程有一定的相关性。

Objective This study was aimed to observe the clinical and pathological feature of myelofibrosis,and to investigate the relationship between such pathological changes and the course and prognosis in hematological diseases. Methods One hundred patients with myelofibrosis（ MF） were retrospectively analyzed,and the pathological features especially the changes of fibrous tissue and vascular system in bone marrow mircroenvironment were reanalyzed. Results There were positive inordinately in all Gomori staining,there were 15 cases of primary myelofibrosis（ PMF） and 85 cases of secondary myelofibrosis（ SMF）. The etiological analysis shows that chronic myelocytic leukemia（ CML）,multiple myeloma（ MM）,essential thrombocythemia（ ET） and MF frequently occur in combinationg.The degree of fibrosis is mild with SMF,and the proliferative degree of fibrosis tissue will decrease after the treatment of primary disease. The Gomori staining were strong positive in most of PMF,and there were low remission after the treatment for improving microenvirenment. Conclusion The degree of fibrosis of PMF is severer than SMF. There is correlation to some degree between SMF and disease course.