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一名患有III型先天性红细胞生成异常性贫血(CDA)患者的多核及巨大单核成红细胞生物学的某些方面。

Some aspects of the biology of multinucleate and giant mononucleate erythroblasts in a patient with CDA, type III.

作者信息

Wickramasinghe S N, Goudsmit R

出版信息

Br J Haematol. 1979 Apr;41(4):485-95. doi: 10.1111/j.1365-2141.1979.tb05886.x.

DOI:10.1111/j.1365-2141.1979.tb05886.x
PMID:435398
Abstract

Marrow cells of a patient with CDA, type III, were (1) incubated with 3H-thymidine for 0.5 h and studied using a combination of Feulgen microspectrophotometry and light microscope autoradiography and (2) incubated with 3H-thymidine, 3H-uridine or 3H-leucine for 1 h and studied using the technique of electron microscope autoradiography. The data revealed multiple abnormalities in the proliferation of erythroblasts but no abnormality in the distribution of neutrophil promyelocytes and myelocytes in the different stages of interphase. Some mononucleate erythroblasts had DNA contents of 4-20c and the multinucleate erythroblasts had total DNA contents of 2-40c. In about 40% of the multinucleate erythroblasts, only some of the nuclei within the same cell incorporated 3H-thymidine and the electron microscope autoradiographic studies revealed that the nuclei which failed to synthesize DNA virtually always showed abnormalities in the electron-density of the heterochromatin or euchromatin or a Swiss-cheese appearance of the heterochromatin. Furthermore, in some multinucleate cells but not in others, the ultrastructurally abnormal nuclei showed a marked depression of RNA synthesis when compared with the normal-looking nuclei within the same cell. An occasional binucleate, multinucleate and giant mononucleate erythroblast showed ultrastructural changes suggestive of advanced degeneration and such cells, which showed a marked depression of RNA and protein synthesis, appeared to be phagocytosed by macrophages.

摘要

一名III型先天性红细胞生成异常性贫血(CDA)患者的骨髓细胞,(1) 用3H-胸腺嘧啶核苷孵育0.5小时,然后结合福尔根显微分光光度法和光学显微镜放射自显影术进行研究;(2) 用3H-胸腺嘧啶核苷、3H-尿嘧啶核苷或3H-亮氨酸孵育1小时,然后采用电子显微镜放射自显影技术进行研究。数据显示幼红细胞增殖存在多种异常,但中性早幼粒细胞和中幼粒细胞在不同间期阶段的分布没有异常。一些单核幼红细胞的DNA含量为4 - 20c,多核幼红细胞的总DNA含量为2 - 40c。在约40%的多核幼红细胞中,同一细胞内只有部分细胞核掺入了3H-胸腺嘧啶核苷,电子显微镜放射自显影研究显示,未能合成DNA的细胞核实际上总是在异染色质或常染色质的电子密度上出现异常,或者异染色质呈现出“瑞士奶酪”样外观。此外,在一些多核细胞而非其他细胞中,与同一细胞内外观正常的细胞核相比,超微结构异常的细胞核显示出RNA合成明显受抑制。偶尔可见的双核、多核和巨大单核幼红细胞呈现出提示晚期退变的超微结构变化,这些细胞显示出RNA和蛋白质合成明显受抑制,似乎被巨噬细胞吞噬。

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1
Some aspects of the biology of multinucleate and giant mononucleate erythroblasts in a patient with CDA, type III.一名患有III型先天性红细胞生成异常性贫血(CDA)患者的多核及巨大单核成红细胞生物学的某些方面。
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引用本文的文献

1
A new case of congenital dyserythropoietic anaemia, type III: studies of the cell cycle distribution and ultrastructure of erythroblasts and of nucleic acid synthesis in marrow cells.一例新的III型先天性红细胞生成异常性贫血:对成红细胞的细胞周期分布、超微结构及骨髓细胞核酸合成的研究
J Clin Pathol. 1982 Oct;35(10):1103-9. doi: 10.1136/jcp.35.10.1103.
2
Studies of erythroblast function in congenital dyserythropoietic anaemia, type I: evidence of impaired DNA, RNA, and protein synthesis and unbalanced globin chain synthesis in ultrastructurally abnormal cells.I型先天性红细胞生成异常性贫血中幼红细胞功能的研究:超微结构异常细胞中DNA、RNA和蛋白质合成受损及珠蛋白链合成失衡的证据。
J Clin Pathol. 1986 Aug;39(8):881-90. doi: 10.1136/jcp.39.8.881.