一名日本人的血小板环氧化酶缺乏症

Platelet cyclo-oxygenase deficiency in a Japanese.

作者信息

Nakanishi K, Ikeda K, Hato T, Imai A, Kaneko H, Murakami A, Kuwashima K, Kaido H, Kondo M, Hattori A

出版信息

Scand J Haematol. 1984 Feb;32(2):167-74. doi: 10.1111/j.1600-0609.1984.tb02173.x.

DOI:10.1111/j.1600-0609.1984.tb02173.x

Abstract

A case of platelet cyclo-oxygenase deficiency in a Japanese was investigated. There was a marked decrease of aggregation with collagen and absence of aggregation with epinephrine and arachidonic acid. The platelet response to a labile aggregation stimulating substance (LASS) was normal. There was no biosynthesis of prostaglandin endoperoxides from arachidonate. The platelets, including granular volume, showed no ultrastructural abnormalities. The responses to various inducers of platelet aggregation, except for arachidonate, were different from the cases described by others. It is concluded that the defective platelet function, due to a deficiency of platelet cyclo-oxygenase, is heterogeneous.

摘要

对一名日本血小板环氧化酶缺乏症患者进行了调查。该患者血小板与胶原蛋白的聚集显著减少，对肾上腺素和花生四烯酸无聚集反应。血小板对不稳定聚集刺激物质（LASS）的反应正常。花生四烯酸不能生物合成前列腺素内过氧化物。包括颗粒体积在内的血小板无超微结构异常。除花生四烯酸外，血小板对各种诱导剂的反应与其他人描述的病例不同。结论是，由于血小板环氧化酶缺乏导致的血小板功能缺陷具有异质性。