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对一名III型先天性红细胞生成异常性贫血患者的成红细胞进行长期电影显微镜检查:发育异常的成红细胞形成的直接观察

Long-term cinemicrography of erythroblasts from a patient with congenital dyserythropoietic anemia type III: direct observation of dysplastic erythroblast formation.

作者信息

Furukawa T, Inoue H, Sugita K, Eguchi M, Sakakibara H, Sugiyama S, Suda T

机构信息

Second Department of Pediatrics, Dokkyo University School of Medicine, Tochigiken, Japan.

出版信息

Blood Cells. 1993;19(2):493-506; discussion 507-8.

PMID:8312576
Abstract

Erythroblasts from a patient with congenital dyserythropoietic anemia type III (CDA III) were observed by time-lapse phase-contrast cinemicrography. The erythroblasts were compressed with a new mechanical device; this procedure enhanced the visualization of their structures. Soret band micrography was carried out simultaneously to assess cytoplasmic hemoglobinization. The proliferation and differentiation course of the majority of CDA III erythroblasts was similar to that of normal erythroblasts. The formation of dysplastic erythroblasts showed two patterns of progression: (1) Usually, the process began with the appearance of a well-demarcated clear area in the nucleus of immature erythroblasts. This clear area reappeared in the progeny of the immature erythroblasts, at an increased incidence and in increasing size and rigidity. It finally came to occupy most of the nucleus, compartmentalizing it. The maturing cells displayed vigorous movement and progression of hemoglobinization and the nuclei became lobular; they grew to the cell size at which most CDA III erythroblasts divide, but did not do so. (2) Less frequently seen were abnormalities in mitosis and cellular behavior (e.g., tri- or bi-polar mitosis and subsequent fusion of daughter cells) or rapid growth of one daughter cell with concomitant shrinkage of the other.

摘要

通过延时相差电影显微镜观察了一名III型先天性红细胞生成异常性贫血(CDA III)患者的成红细胞。用一种新的机械设备对成红细胞进行挤压;该操作增强了其结构的可视化效果。同时进行索雷特带显微摄影以评估细胞质血红蛋白化情况。大多数CDA III成红细胞的增殖和分化过程与正常成红细胞相似。发育异常的成红细胞的形成表现出两种进展模式:(1)通常,该过程始于未成熟成红细胞细胞核中出现界限清晰的透明区域。这个透明区域在未成熟成红细胞的子代中再次出现,发生率增加,且大小和硬度不断增大。它最终占据了大部分细胞核,将其分隔开来。成熟细胞表现出活跃的运动以及血红蛋白化进程,细胞核变成小叶状;它们生长到大多数CDA III成红细胞分裂时的细胞大小,但并未进行分裂。(2)较少见的情况是有丝分裂和细胞行为异常(例如,三极或双极有丝分裂以及随后子细胞的融合),或者一个子细胞快速生长而另一个子细胞同时萎缩。

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Congenital dyserythropoietic anaemia (CDA) type I--a case report with ultrastructural study.
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