FAMILIAL AND CONSTITUTIONAL BLEEDING DISORDER DUE TO PLATELET CYCLO-OXYGENASE DEFICIENCY
FAMILIAL AND CONSTITUTIONAL BLEEDING DISORDER DUE TO PLATELET CYCLO-OXYGENASE DEFICIENCY
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DOI：
10.1002/ajh.2830140102
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发表时间：
1983-01-01
影响因子：
12.8
通讯作者：
SAMAMA, M
中科院分区：
文献类型：
--
作者：
HORELLOU, MH;LECOMPTE, T;SAMAMA, M
连续两代的三名家庭成员有出血倾向。他们的模板出血时间延长，ADP和肾上腺素诱导的血小板聚集没有显示第二波;低至中等浓度的胶原蛋白不能聚集和释放ATP，而较高的量聚集和释放。凝血酶、利托那肽和合成环氧衍生物（U 44069和U 44619）引起的聚集和释放正常。花生四烯酸（AA）是无活性的，在兔主动脉条上评价的血栓烷（TX）A2活性中，也没有转化，通过放射免疫测定法和放射色谱法评价的TXB 2 [血栓烷]也没有转化。患者血小板对TXB 2和PGE 2 [前列腺素E2]形成的平行损害与血小板环氧合酶缺乏症相一致。传播为常染色体显性遗传。环氧合酶对于大量胶原蛋白的聚集和ATP释放是不需要的。
Three family members from 2 successive generations had a bleeding tendency. Their template bleeding time was prolonged and platelet aggregation induced by ADP and adrenaline [epinephrine] showed no 2nd wave; collagen at low to moderate concentrations failed to aggregate and release ATP, whereas higher amounts aggregated and released. Aggregation and release due to thrombin, ristocetin and synthetic epoxy derivatives (U 44069 and U 44619) were normal. Arachidonate (AA) was inactive and was not converted either in thromboxane (TX) A2 activity evaluated on the rabbit aorta strip, nor in TXB2 [thromboxane] evaluated by radioimmunoassay and by radiochromatography. The parallel impairment of TXB2 and PGE2 [prostaglandin E2] formation by the patient''s platelets are compatible with a platelet cyclooxygenase deficiency. Transmission is autosomal dominant. Cyclooxygenase is not needed for aggregation and ATP release by high amounts of collagen.