Static and functional morphology of the pathological platelets in primary myelofibrosis and myeloproliferative syndrome.

Series haematologica (1968) Pub Date : 1975-01-01
A Hattori, K Koike, S Ito, M Matsuoka
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Abstract

Native and functioning platelets in patients with primary myelofibrosis and myeloproliferative syndrome were examined by transmission electron microscopy. Among various types of ultrastructural abnormalities in the platelets, three features were emphasized: hypoplasia of the surface connecting system (SCS) with few orifices, hyperplasia of the dense tubular system, and considerable variety in numbers of granule. Morphological analysis was made on platelets of these patients and of normal subjects. Functional morphology of the abnormal platelets was examined in the aggregate samples either by ADP or collagen and the effluent blood from platelet retention test. The abnormal platelets were more or less indifferent from the aggregates and underwent only to a slight degree the changes observed in normal platelets. They frequently retained their native, round, smooth-surfaced form without pseudpod, and did not show the inward shift of organelles; furthermore, they retained more granules than did normal platelets. From these morphological findings we speculated that these ultrastructurally abnormal platelets had an impaired release reaction as a result of the dysfunction of SCS in passing released substances, of the inability of microfilaments to constrict in expelling these substances and sometimes because of a lack of granules as the sources.

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原发性骨髓纤维化和骨髓增生性综合征病理血小板的静态和功能形态。
透射电镜观察原发性骨髓纤维化和骨髓增生性综合征患者的天然血小板和功能血小板。在各种类型的血小板超微结构异常中,强调了三个特征:表面连接系统(SCS)发育不全,孔口较少,致密管状系统增生,颗粒数量变化很大。对这些患者和正常人的血小板进行形态学分析。用ADP法、胶原法和血小板保留试验排出血法检测凝集标本中异常血小板的功能形态。异常血小板或多或少与聚集体无关,只经历了正常血小板中观察到的轻微程度的变化。它们经常保持原来的圆的、表面光滑的形状,没有假足,也没有显示出细胞器向内移动;此外,它们比正常血小板保留了更多的颗粒。根据这些形态学发现,我们推测这些超微结构异常的血小板释放反应受损,这是由于SCS通过释放物质的功能障碍,微丝无法收缩以排出这些物质,有时是因为缺乏颗粒作为来源。
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The ultrastructure of the platelets in refractory anemia ("preleukemia") and myelomonocytic leukemia. Heterogeneity of megakaryocytes and platelets. Development of two distinct membrane systems associated in giant complexes in pathological megakaryocytes. Classification and possible mechanisms of action of some drugs that inhibit platelet aggregation. Inhibitors of platelet function in the prevention of arterial thrombosis.
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