广西医科大学学报2017，Vol.34Issue(6)：810-813,4.DOI:10.16190/j.cnki.45-1211/r.2017.06.003
MYH9相关综合征患者血小板和中性粒细胞的超微结构及其病理特点
Ultrastructure and pathologic features of platelet and neutrophil in patients with MYH9-RD
摘要
Abstract
Objective:To study the ultrastructure and pathologic features of platelet and neutrophil in patients from a family (GXMS family) with non-muscle myosin heavy chain 9 related disease (MYH9-RD).Methods:Morphological characteristics of neutrophils,platelets and their organelles in peripheral blood of patients were observed by optical microscope and transmission electron microscope.Results:The proband in the GXMS family presented with giant platelet,thrombocytopenias and neutrophil inclusions.The platelets of all patients were heterogeneous in organelles content,and had increasing number of dense and α-granules,as well as open-canalicular systems,which were proportional to the increased platelet volume.The mitochondrial swelled significantly in 85％ of the platelets,the open-tubular increased obviously in 62％ of the platelets,and the pseudopodia were observed in 90％ of the platelets.Some regions in giant platelets were surrounded by microtubules and endoplasmic reticulum,which were similar to the neutrophil inclusions in shape.Conclusion:Abnormal NMMHC-ⅡA resulted in a inhomogeneous organelle distribution within platelets,and it is likely to be an essential factor for maintaining the activation state of the platelets in patients with MYH9-RD.关键词
MYH9相关综合征/血小板减少症/透射电子显微镜/巨大血小板/中性粒细胞包涵体Key words
MYH9-related disorders/macrothrombocytopenia/transmission electron microscopy/giant platelet/neutrophil inclusion body分类
医药卫生引用本文复制引用
廖文君,罗晓成,吴华裕,舒伟,张学,陈萍,袁志刚..MYH9相关综合征患者血小板和中性粒细胞的超微结构及其病理特点[J].广西医科大学学报,2017,34(6):810-813,4.基金项目
国家自然科学基金资助项目(No.30860125) （No.30860125）
广西科技计划基地和人才专项重点实验室建设资助项目(No.16-380-34) （No.16-380-34）
