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原发性(特发性)血小板增多症患者人骨髓中巨核细胞的超微结构

Ultrastructure of megakaryocytes in the human bone marrow of patients with primary (essential) thrombocythemia.

作者信息

Thiele J, Jansen B, Orth K H, Orth H, Moedder B, Fischer R

机构信息

Institute of Pathology, University of Cologne, West Germany.

出版信息

J Submicrosc Cytol Pathol. 1988 Oct;20(4):671-81.

PMID:3224337
Abstract

An ultrastructural study was performed on bone marrow specimens in 10 patients (5 males/5 females, median age 53 years) with primary (essential) thrombocythemia (PTH) and an excessive elevation of the platelet count (1,625 +/- 783 x 10(9)/l). In contrast to a not severely altered neutrophilic granulo- and erythrocytopoiesis, megakaryocytes showed conspicuous large to giant forms. These were characterized by a highly lobulated nucleus containing several nucleoli and an extensive intermediate zone of the cytoplasm with many Golgi fields, numerous profiles of the so-called demarcation membrane system and an abundance of alpha-granules and some dense bodies. Our results demonstrate that ultrastructure of the megakaryocytes in PTH does not reveal gross abnormalities, but features which are compatible with an enforced thrombocytogenetic activity in accordance with the excessively elevated platelet count. Similar changes have been described in animal experiments with induced thrombocytopenia and stimulation of platelet shedding. Evaluation of thrombocytogenesis suggests that it may be mediated by a process of fragmentation with partitioning of the extensive intermediate zone into numerous prospective platelet territories followed by segregation.

摘要

对10例原发性(特发性)血小板增多症(PTH)且血小板计数极度升高(1,625±783×10⁹/L)的患者(5例男性/5例女性,中位年龄53岁)的骨髓标本进行了超微结构研究。与嗜中性粒细胞生成和红细胞生成未严重改变形成对比的是,巨核细胞呈现出明显的大到巨型形态。这些巨核细胞的特征是细胞核高度分叶,含有多个核仁,细胞质的中间区域广泛,有许多高尔基体区域、大量所谓的分界膜系统轮廓以及丰富的α颗粒和一些致密小体。我们的结果表明,PTH中巨核细胞的超微结构未显示明显异常,但其特征与根据血小板计数极度升高而增强的血小板生成活性相符。在诱导血小板减少和刺激血小板释放的动物实验中也描述了类似的变化。对血小板生成的评估表明,它可能由一个分裂过程介导,即将广泛的中间区域分隔成许多预期的血小板区域,随后进行分离。

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