Bellucci S, Kedra A, Bray P, Caen J P
Department of Angiohematology, Lariboisiere Hospital, Paris, France.
Angiology. 1990 Nov;41(11):970-2. doi: 10.1177/000331979004101112.
"Raynaud's phenomenon (R.P.) is characterized by a spastic vasoconstriction of hand and/or feet extremities leading to painful ischemic attacks. In the pathogeny of this syndrome the role of a platelet hyperactivation was evidenced. Nevertheless, the responsibility of platelet aggregation remains controversial. We report here the case of a patient presenting with a Glanzmann thrombasthenia defined by the complete lack of platelet aggregation, and showing a typical RP which was confirmed by the measurement of hand temperature and the decrease of digital and hand blood flow. Thus, the description of this case shows that platelet aggregation by itself is not necessary for the occurrence of RP, and encourages for therapeutic purposes the development of vasodilatators rather than platelet antiaggregants agents."
雷诺现象(R.P.)的特征是手足末端发生痉挛性血管收缩,导致疼痛性缺血发作。在该综合征的发病机制中,血小板过度活化的作用已得到证实。然而,血小板聚集的责任仍存在争议。我们在此报告一例患有Glanzmann血小板无力症的患者,其表现为完全缺乏血小板聚集,并呈现典型的雷诺现象,这通过手部温度测量以及手指和手部血流减少得到证实。因此,该病例的描述表明,血小板聚集本身并非雷诺现象发生所必需,这为治疗目的鼓励开发血管扩张剂而非血小板抗聚集剂。