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由于血小板膜复合物缺陷导致的血小板病。

Thrombocytopathy due to a defect of the platelet membrane complex.

作者信息

Canizares C, Vivar N, Grijalva J

机构信息

Department of Hematology, Hospital Andrade Marin, IESS, Quito, Ecuador.

出版信息

Acta Haematol. 1990;83(2):99-104. doi: 10.1159/000205178.

DOI:10.1159/000205178
PMID:2106203
Abstract

A new type of primary thrombocytopathy is described. Three main alterations were found: (1) a defect of the aggregation reaction with ADP, epinephrine and collagen and a normal response to ristocetin and arachidonic acid; (2) a moderate deficiency of platelet procoagulant activity, and (3) a combined hypertrophy of the two membrane systems of the platelet--the open canalicular and the dense tubular. The latter defect is shown as an abnormal membrane complex situated on one of the platelet poles. This thrombocytopathy is discussed as a new variety of primary platelet disorder.

摘要

本文描述了一种新型原发性血小板病。发现了三个主要改变:(1)对ADP、肾上腺素和胶原的聚集反应缺陷,而对瑞斯托菌素和花生四烯酸反应正常;(2)血小板促凝活性中度缺乏;(3)血小板的两个膜系统——开放小管系统和致密小管系统联合肥大。后一种缺陷表现为位于血小板一极的异常膜复合物。本文将这种血小板病作为原发性血小板疾病的一种新类型进行了讨论。

相似文献

1
Thrombocytopathy due to a defect of the platelet membrane complex.由于血小板膜复合物缺陷导致的血小板病。
Acta Haematol. 1990;83(2):99-104. doi: 10.1159/000205178.
2
Haemorrhagic thrombocytopathy associated with dilatation of the platelet--membrane complex.与血小板-膜复合物扩张相关的出血性血小板病
Br J Haematol. 1981 Aug;48(4):595-600. doi: 10.1111/j.1365-2141.1981.00595.x.
3
[Aspirin-like defect - a hereditary thrombocytopathy due to impaired release of platelet adenosine diphosphate].[阿司匹林样缺陷——一种因血小板二磷酸腺苷释放受损导致的遗传性血小板病]
Bilt Hematol Transfuz. 1979;7(2-3):165-74.
4
Failure to mobilize intracellular calcium in response to thrombin in a patient with familial thrombocytopathy characterized by macrothrombocytopenia and abnormal platelet membrane complexes.在一名以大血小板减少和异常血小板膜复合物为特征的家族性血小板病患者中,对凝血酶反应时细胞内钙动员失败。
J Lab Clin Med. 1993 Oct;122(4):441-9.
5
Hereditary thrombocytopathy: a familial bleeding disorder due to impaired platelet coagulant activity.遗传性血小板病:一种由于血小板凝血活性受损导致的家族性出血性疾病。
Aust N Z J Med. 1976 Feb;6(1):37-45. doi: 10.1111/j.1445-5994.1976.tb03289.x.
6
Use of scanning electron microscopy to study structural-functional relationships in normal and diseased platelets.使用扫描电子显微镜研究正常和病变血小板的结构-功能关系。
Scan Electron Microsc. 1985(Pt 1):357-91.
7
[The platelet defect in myeloproliferative disorder (author's transl)].
Nouv Rev Fr Hematol (1978). 1976;16(3):443-6.
8
Association of the hemophilia A carrier state and hemorrhagic thrombocytopathy with dilatation of the platelet membrane complex.甲型血友病携带者状态及出血性血小板病与血小板膜复合物扩张的关联。
Acta Haematol. 1984;71(6):381-7. doi: 10.1159/000206623.
9
The platelet: membrane and surface reactions.血小板:膜与表面反应
Annu Rev Physiol. 1976;38:501-35. doi: 10.1146/annurev.ph.38.030176.002441.
10
Essential athrombia: study of a new case.原发性无血栓形成:1例新病例研究
Thromb Diath Haemorrh. 1975 Apr 30;33(2):278-85.

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