Lagarde M, Byron P A, Vargaftig B B, Dechavanne M
Br J Haematol. 1978 Feb;38(2):251-66. doi: 10.1111/j.1365-2141.1978.tb01041.x.
Two cases of thrombocytopathia with congenital deficiency of platelet cyclo-oxygenase were investigated. The platelet release reaction was impaired. There was a marked decrease of aggregation with collagen and with adrenalin and a total absence of aggregation with sodium arachidonate. The platelet response to labile aggregation stimulating substance (LASS, mostly thromboxane A2) was normal. There was no biosynthesis of prostaglandin cyclic endoperoxides or of thromboxane A2 from arachidonic acid. Basal levels of platelet PGE1 were lowered although plasma levels were normal. Thrombin decreased the cyclic AMP content of patients' platelets and also that of control platelets pretreated with aspirin. The patients platelets showed no ultrastructural difference when compared with control platelets, except for a slight decrease of granule volume, but, in contrast to control platelets, thrombin (0.02 U/ml) did not provoke contraction of the patients' platelets.
对两例先天性血小板环氧化酶缺乏所致血小板病进行了研究。血小板释放反应受损。与胶原蛋白和肾上腺素的聚集明显减少，与花生四烯酸钠完全不聚集。血小板对不稳定聚集刺激物质（LASS，主要是血栓素A2）的反应正常。花生四烯酸不能生物合成前列腺素环内过氧化物或血栓素A2。尽管血浆水平正常，但血小板PGE1的基础水平降低。凝血酶降低了患者血小板以及用阿司匹林预处理的对照血小板的环磷酸腺苷含量。与对照血小板相比，患者血小板除颗粒体积略有减小外，未显示超微结构差异，但与对照血小板不同的是，凝血酶（0.02 U/ml）未引起患者血小板收缩。
