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慢性巨核细胞-粒细胞性骨髓增殖症——一项电镜研究。I. 巨核细胞和血小板。

Chronic megakaryocytic-granulocytic myelosis--an electron microscopic study. I. Megakaryocytes and thrombocytes.

作者信息

Thiele J, Ballard A C, Georgii A, Vykoupil K F

出版信息

Virchows Arch A Pathol Anat Histol. 1977 Apr 6;373(3):191-211. doi: 10.1007/BF00432237.

Abstract

The fine structure of the bone marrow in chronic megakaryocytic-granulocytic myelosis (CMGM) was studied in 5 nontreated patients to investigate possible malignant proliferation of megakaryocytes and the role of megakaryopoiesis in fibrillogenesis, terminating in osteomyelofibrosis. In comparison with normal megakaryopoiesis there is an enormous increase of the megakaryocytic cell line and many immature and atypical forms are seen. Most conspicuous are microforms, nuclear-cytoplasmic disorganization and nuclear inclusions. Asynchrony of maturation causes abnormal thrombocytogenesis with premature detachment of platelets resulting in immature and peculiar giant forms of thrombocytes. Besides megakaryocytes appearing superficially normal the maturation anarchy of many cells is so severe that by analogy with observations in other leukaemic cells these abnormalities are thought to be representative of a malignant growth. Moreover, there is a striking accumulation of microfibrils and single collagen fibres around megakaryoblasts. Since these cells contain all those organelles commonly associated with fibre production the initial step for fibrillogenesis may therefore arise from the megakaryoblasts prior to platelet release, or any fibroblast proliferation.

摘要

对5例未经治疗的慢性巨核细胞 - 粒细胞性骨髓化生(CMGM)患者的骨髓精细结构进行了研究,以探讨巨核细胞可能的恶性增殖以及巨核细胞生成在纤维形成(最终导致骨髓纤维化)中的作用。与正常巨核细胞生成相比,巨核细胞系大量增加,可见许多不成熟和非典型的形态。最明显的是微小形态、核质紊乱和核内包涵体。成熟不同步导致血小板生成异常,血小板过早脱离,产生不成熟和奇特的巨大血小板形态。除了表面看似正常的巨核细胞外,许多细胞的成熟紊乱非常严重,根据在其他白血病细胞中的观察结果类推,这些异常被认为代表恶性生长。此外,在原始巨核细胞周围有明显的微纤维和单条胶原纤维堆积。由于这些细胞含有所有通常与纤维产生相关的细胞器,因此纤维形成的初始步骤可能在血小板释放之前由原始巨核细胞产生,而不是任何成纤维细胞增殖。

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