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真性红细胞增多症:10例未经治疗患者骨髓的电子显微镜检查。薄切片与冷冻断裂研究。

Polycythemia vera: electron microscopy of the bone marrow in 10 non-treated patients. A thin section and freeze-fracture study.

作者信息

Thiele J, Stangel W, Vykoupil K F, Georgii A

出版信息

Blut. 1979 May;38(5):407-20. doi: 10.1007/BF01007902.

Abstract

Electron microscopy (thin sections and freeze-fracture replicas) was performed on the bone marrow of ten patients with Polycythemia vera prior to any treatment. In addition to a hyperplasia of all three cell lineages and the sinuses, atypias were observed in the maturing erythroblasts. These aberrations of normal development consisted mainly of deep invaginations of the nuclear envelope in proerythroblasts and conspicuous nuclear clefts in erythro- and normoblasts. In comparison with similar changes in dyserythropoietic and aplastic anemia as well as leukemia these alterations are discussed in connection with disturbances of DNA synthesis. Further atypias involved megakaryopoiesis which displayed microforms probably as an evidence for maturation arrest. These ultrastructural abnormalities and their morphological features of a neoplastic proliferation of all three cell lineages in Polycythemia vera are in good agreement with the new concept of a transformation of a pluripotent stem cell with clonal character.

摘要

对10例真性红细胞增多症患者在任何治疗之前的骨髓进行了电子显微镜检查(薄切片和冷冻断裂复制品)。除了所有三个细胞系和血窦增生外,在成熟的成红细胞中观察到异型性。正常发育的这些异常主要包括早幼红细胞中核膜的深内陷以及晚幼红细胞和正成红细胞中明显的核裂。与骨髓异常增生性贫血、再生障碍性贫血以及白血病中的类似变化相比,这些改变与DNA合成障碍有关。进一步的异型性涉及巨核细胞生成,其显示出微形态,可能是成熟停滞的证据。真性红细胞增多症中所有三个细胞系的这些超微结构异常及其肿瘤性增殖的形态学特征与具有克隆特征的多能干细胞转化的新概念高度一致。

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