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本研究探讨Ⅰ型先天性红细胞生成不良性贫血(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。
This study was to investigate the ultrastructural features of type Ⅰ congenital erythropathy (CDA-Ⅰ) and the main points of electron microscopy. Transmission electron microscopy was used to analyze the ultrastructure of nucleated erythrocytes in 2 children. The results showed that the percentage of erythroblasts in two types of CDA-Ⅰ patients was increased, and the cells in different stages were different in size. The nuclei of erythrocytes were irregular, the nuclei of cheese were observed in young or middle red blood cells, Dissolution and fragmentation, late karyonuclear injury sometimes accompanied by cytoplasmic lysis, interstitial chromatin bridge rare; erythrocyte nuclear membrane and endoplasmic reticulum at different stages of different degrees of dissolution at the same time. CONCLUSION: The main ultrastructural features of CDA-Ⅰ type are megaloblastic hyperplasia of erythroblasts, followed by nuclear and nuclear fragmentation at middle and young stages and at late and juvenile stages. The extensive destruction of biofilm system is CDA-Ⅰ The main pathology.