Mammen E F
Wayne State University School of Medicine, Detroit, Michigan, USA.
Semin Thromb Hemost. 1999;25(4):361-5. doi: 10.1055/s-2007-994939.
The sticky platelet syndrome (SPS) is an autosomal dominant platelet disorder associated with arterial and venous thromboembolic events. It is characterized by hyperaggregability of platelets in platelet-rich plasma with adenosine diphosphate (ADP) and epinephrine (type I), epinephrine alone (type II), or ADP alone (type III). Clinically, patients may present with angina pectoris, acute myocardial infarction (MI), transient cerebral ischemic attacks, stroke, retinal thrombosis, peripheral arterial thrombosis, and venous thrombosis, frequently recurrent under oral anticoagulant therapy. Clinical symptoms, especially arterial, often present following emotional stress. Combinations of SPS with other congenital thrombophilic defects have been described. Low-dose aspirin treatment (80 to 100 mg) ameliorates the clinical symptoms and normalizes hyperaggregability. The precise etiology of this defect is at present not known, but receptors on the platelet surface may be involved. Normal levels of platelet factor 4 (PF4) and beta-thromboglobulin in plasma suggest that the platelets are not activated at all times; they appear to become hyperactive upon ADP or adrenaline release. In vivo clumping could temporarily or permanently occlude a vessel, leading to the described clinical manifestations. The syndrome appears to be prominent especially in patients with unexplained arterial vascular occlusions.
粘性血小板综合征(SPS)是一种常染色体显性遗传性血小板疾病,与动脉和静脉血栓栓塞事件相关。其特征是富含血小板的血浆中的血小板在二磷酸腺苷(ADP)和肾上腺素作用下(I型)、仅在肾上腺素作用下(II型)或仅在ADP作用下(III型)具有高聚集性。临床上,患者可能出现心绞痛、急性心肌梗死(MI)、短暂性脑缺血发作、中风、视网膜血栓形成、外周动脉血栓形成和静脉血栓形成,在口服抗凝治疗下常反复发生。临床症状,尤其是动脉症状,常在情绪应激后出现。已报道SPS与其他先天性血栓形成缺陷的组合。低剂量阿司匹林治疗(80至100毫克)可改善临床症状并使高聚集性恢复正常。目前尚不清楚这种缺陷的确切病因,但可能涉及血小板表面的受体。血浆中血小板因子4(PF4)和β-血小板球蛋白水平正常表明血小板并非一直处于激活状态;它们似乎在ADP或肾上腺素释放时变得过度活跃。体内聚集可暂时或永久性阻塞血管,导致上述临床表现。该综合征在患有不明原因动脉血管闭塞的患者中似乎尤为突出。