先天性红细胞生成障碍贫血I型的超微结构特征_茹永新.pdfVIP

中国实验 液学杂志　Journal of Exp ermi en talH emato logy 2007;15 (1 ):117 -120 117 (Article ID):1009 -2137(2007)01 -0117 -04 I 茹永新, 竺晓凡, 赵轼轩, 刘津华,钟舒 中国医学科学院 中国协和医科大学 血液学研究所 血液病医院,天津3 00020 　　本研究探讨Ⅰ型先天性红细胞生成不良性贫 (CDA-Ⅰ型)的超微病理特点和电镜诊断要点。用透射电 子显微镜分析 2例儿童患者骨髓有核红细胞的超微结构。结果表明:2例 CDA-Ⅰ型患者骨髓 幼红细胞比例增加, 各阶段细胞存在不同程度巨幼样变;原红细胞核不规则, 早幼或中幼红细胞可见奶酪核, 约半数晚幼红细胞有核溶 解和破碎现象, 晚幼红细胞核损伤有时伴胞质溶解, 细胞间染色质桥少见;各阶段红细胞核膜和内质网同时出现不 同程度溶解。结论:CDA-Ⅰ型的主要超微结构特点为幼红细胞巨幼样增生, 其次是中幼阶段核膜损伤和晚幼阶段 的核溶解和核碎裂, 生物膜系统广泛破坏是 CDA-Ⅰ型主病理机制。 　　贫 ;先天性红细胞生成障碍性贫 Ⅰ型;成红细胞;超微结构 　　R556. 9 　　A U ltrastructura l Character istics of Congenital Dy sery th ropoietic A nem ia- type I RU Yong-X in,ZH U Xiao-Fan,ZHAO Shi-Yuan,LIU Jin-Hua,ZHONG Shu Departmen t of E le tron M i ros opy, Institute of H ema tology and B lood D iseasesH ospital, Peking Un ion Medi a lCo llege, Ch in ese A- adem y ofM ed i a l S ien es, Tianj in, 300020, Ch ina Abstract　　The study was a im ed to inves tiga te the ultran struc tural feature and diagnostic cr ite ria of congen ita l dyserythrop oietic anem ia-typ e I (CDA-typ e Ⅰ ). Nucleated red cells in bone m arrow from two patien ts w ith CDA-type Ⅰ we re analyzed by tran sm ission electron m icroscopy (TEM ). The results indicated that the erythropo ie tic granulopo ie tic ra tio w as m arked ly increased w ith m ega lob lastic m orph ology in all stage of erythrocyte. M ost proerythroblast sh owed of irregular nuc le,i w hile the Sw iss-cheese-app earance of the he terochrom atin w as u sually found in basophilic and p olychrom a tic erythroblas.t Ab ou t ha lf of orth ochrom atic erythroblast illustrated karyo ly sis and karyorrhex is. Som e or thoch rom atic erythroblast exh ib ited karyolys is and p lasm o ly sis sim u ltaneou sly.