非肌球蛋白重链9相关疾病大家系患者中性粒细胞包含体特点分析  

Analysis of neutrophils inclusions for the patients from a nonmuscle myosin heavy chain 9 related disease family

作　　者：张淑芳[1,2] 张应爱[1,2] 王顺兰[1,2] 肖敬川[1,2] 黄邓高[1,2] 

机构地区：[1]海口市人民医院中心实验室 [2]中南大学湘雅医学院附属海口医院,海南海口570208

出　　处：《中国实验诊断学》2013年第1期23-26,共4页Chinese Journal of Laboratory Diagnosis

基　　金：海南省科技厅自然科学基金资助课题(806104;309114);海南省卫生厅科研基金资助课题(2009-72;2010-60);海口市科技局重点科技资助课题(2006-0020;2008-0000207;2009-049-2)

摘　　要：目的分析一非肌球蛋白重链9相关疾病(MYH9-RD)大家系患者中性粒细胞包涵体的形态和结构特点,阐明MYH9-RD中性粒细胞包涵体形成的特点,以探讨MYH9-RD中性粒细胞包涵体的致病机制。方法应用光学显微镜(瑞姬染色)和电子显微镜观察中性粒细胞包涵体形态和超微结构特点;应用间接免疫荧光复染技术观察MYH9-RD患者与正常对照者之间非肌性肌球蛋白IIA(NMMHC-IIA)在胞浆中的分布情况。结果光学显微镜和电子显微镜下可见中性粒细胞中明显的包涵体,形态多样,大多位于细胞边缘;间接免疫荧光复染技术显示患者和正常人之间NMMHC-IIA在中性粒细胞中的分布有差异,中性粒细胞胞浆中存在明显绿色荧光的包涵体,其形态和轮廓与瑞一姬氏染色显示的包涵体形状、大小基本一致,但更为清晰;正常对照的粒细胞胞浆中未见包涵体。结论光学显微镜和电子显微镜进一步确认了家系患者包涵体的存在。免疫荧光技术显示患者和正常人之间NMMHC-IIA在中性粒细胞中的分布有差异;MYH9-RD患者中性粒细胞包涵体的主要构成成份是NMMHC-IIA;包涵体的形态结构复杂多样可能与本家系临床表型复杂多样有关,该家系对进一步揭示MYH9-RD的致病机制具有重要的意义。Objective To explore the pathologic mechanism of neutrophils inclusions with nonmuscle myosin heavy chain 9 related disease （MYH9-RD） by observing and analyzing the characteries of strcture and formation of neutro- phils inclusions with MYH9-RD patients. Methods Technique of light microscope（Wright-Giemsa staining） and elec- tron microscope was applied to analyze the cell morphology and ultrastructure of neutrophils inclusions of peripheral blood;Using indirect immunofluorescence technique to observe and analyze the localization and formation of non muscle myosin heavy chain IIA（NMMHC-IIA）. Results The neutrophils inclusion bodies with morphous multiplicity which localized in the periphery of the neutrophils were clearly seen under light microscope and eletron microscope. Mean- while,by immunofluorescence technique, neutrophils inclusions with green fluorescence were markly showed, which matched very well in localization, size and shape, compared with the inclusions ,displayed by light microscope （Wright- Giemsa＇s stain）. However,in normal control, no inclusions were observed except a diffusive distribution of fluorescent spot in neutrophils cytoplasm. Conclusion The neutrophils inclusions were confirmed furtherly by light microscope and eletron microscope. The distribution of NMMHC-IIA in cytoplasm of neutrophils between patients and normal control is different revealed by immunofluorescence technology. The major Component of neutrophils inclusions from MYH9- RD patients are NMMHC-IIA. The diversity of inclusions from the family patients may be related to its complicated clinical manifestations. It is of significance to reveal the molecular pathogenesis mechanism of MYH9-RD by further study this family.

关 键 词：非肌球蛋白重链9相关疾病(MYH9-RD) 中性粒细胞包涵体 非肌性肌球蛋白IIA(NMMHC-IIA) 

分 类 号：R446.8[医药卫生—诊断学]