[伴有环氧化酶缺乏的血小板病]
[Thrombocytopathia with cyclo-oxygenase deficiency].
作者信息
Dechavanne M, Lagarde M, Bryon P A
出版信息
Nouv Rev Fr Hematol (1978). 1976;16(3):421-6.
In four patients, platelet aggregation with arachidonate was absent but was normal with "Labile Aggregation Stimulating Substance". ADP (10muM) aggregation was always reversible; collagen aggregation was absent and those induced by thrombin and ristocetin were normal. Collagen did not induce 14C-serotonin release but there was some release with thrombin. There was no thromboxan B2 synthesis in these platelets. Two patients showed hereditary hemostasis defect and the others acquired cyclo-oxygenase deficiency.
4例患者对花生四烯酸的血小板聚集反应缺失，但对“不稳定聚集刺激物质”的反应正常。ADP（10μM）诱导的聚集反应总是可逆的；对胶原无聚集反应，对凝血酶和瑞斯托霉素诱导的聚集反应正常。胶原不诱导14C-5-羟色胺释放，但凝血酶可诱导一定程度的释放。这些血小板中无血栓素B2合成。2例患者表现为遗传性止血缺陷，其他患者为获得性环氧化酶缺乏。
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