Ru Yong-Xin, Dong Shu-Xu, Li Yuan, Zhao Shi-Xuan, Liang Hao-Yue, Zhu Xiao-Fan, Zheng Yi-Zhou, Zhang Feng-Kui
a State Key Laboratory of Experimental Hematology, Peking Union Medical College , Institute of Hematology & Blood Diseases Hospital , Beijing , China.
Ultrastruct Pathol. 2018 Jul-Aug;42(4):350-357. doi: 10.1080/01913123.2018.1485807. Epub 2018 Jun 18.
Sixteen patients with mild anemia and hemolysis were difficult to be classified into any known category based on laboratory examinations and light microscopy. To make a definite diagnosis and investigate the pathomechanism, ultrastructural study was performed on erythroid cells from 16 patients. Transmission electron microscopy demonstrated a series of alterations of cytoplasm, including cytoplasm sequestration, membranous transformation, and degeneration in erythroblasts and reticulocytes at different stages. The affected erythroblasts were usually complicated with chromatin condensation, karyorrhexis, nuclear membrane lysis, and megaloblastic changes. The reticulocytes with the cytoplasm alterations had a huge size from 10 um to 15 um in diameter. The membranous cytoplasm degeneration revealed a unique pathomechanism of dyserythropoiesis and ineffective erythropoiesis in 16 patients with anemia, and suggested a novel anemia category though more details remained to be investigated.
16例轻度贫血和溶血患者,根据实验室检查和光学显微镜检查结果,难以归入任何已知类型。为了做出明确诊断并研究发病机制,对16例患者的红系细胞进行了超微结构研究。透射电子显微镜显示细胞质有一系列改变,包括不同阶段的成红细胞和网织红细胞中的细胞质隔离、膜性转化和变性。受影响的成红细胞通常伴有染色质凝聚、核碎裂、核膜溶解和巨幼样改变。有细胞质改变的网织红细胞直径巨大,为10微米至15微米。膜性细胞质变性揭示了16例贫血患者红细胞生成异常和无效红细胞生成的独特发病机制,并提示了一种新的贫血类型,尽管更多细节仍有待研究。