Pope J M, Canny C L, Bell D A
Department of Medicine, University of Western Ontario, University Hospital, London, Canada.
Am J Med. 1991 Mar;90(3):299-309.
A group of young patients presenting with cerebral ischemic events, endocardial lesions, and lupus anticoagulant is described in order to highlight the common clinical laboratory features.
Fourteen consecutive patients (10 females, age range 17 to 53 years [mean 38 years]) at onset of symptoms of cerebral ischemia who had evidence of the lupus anticoagulant syndrome and were being followed prospectively are reviewed. All patients had abnormal phospholipid-dependent coagulation test results, and most had anticardiolipin antibody at the time of presentation. Three of 14 had four or more American Rheumatism Association criteria for definite systemic lupus erythematosus and the remaining patients were considered to have primary lupus anticoagulant syndrome.
The common features among these patients included at least one cerebral ischemic event at presentation (stroke or transient ischemic attack), or recurrent episodes suggesting cerebral ischemia (amaurosis fugax, recurrent severe migraine headaches), livedo reticularis, endocardial valvular lesions noted on echocardiography (11 mitral, two aortic valve) that were often associated with discrete vegetations, retinal vascular lesions, and computed tomographic/magnetic resonance imaging scanning or angiographic evidence of multiple cerebral infarction. Venous thromboembolic events were uncommon (three of 14). Common laboratory studies included thrombocytopenia (10 of 14), positive direct Coombs' test result (11 of 14), and hypocomplementemia (11 of 14). Follow-up after initial treatment with either salicylates or anticoagulant therapy (warfarin) for up to 10 years indicated that while many patients had recurrent symptoms suggesting cerebral ischemia, major stroke syndromes did not recur nor new episodes emerge.
The combination of multiple cerebral ischemic lesions and endocardial lesions, including valvular vegetations, suggests that these cerebral ischemic events represent cerebral emboli, and that these cerebral embolic events originate from vegetative lesions on the mitral or, less commonly, aortic valve, in association with lupus anticoagulant.
描述一组出现脑缺血事件、心内膜病变和狼疮抗凝物的年轻患者，以突出其常见的临床实验室特征。
回顾了14例连续的患者（10例女性，年龄范围17至53岁[平均38岁]），这些患者在出现脑缺血症状时具有狼疮抗凝物综合征的证据，并正在接受前瞻性随访。所有患者的磷脂依赖性凝血试验结果均异常，且大多数患者在就诊时存在抗心磷脂抗体。14例患者中有3例符合美国风湿病学会明确的系统性红斑狼疮的四项或更多标准，其余患者被认为患有原发性狼疮抗凝物综合征。
这些患者的共同特征包括就诊时至少发生一次脑缺血事件（中风或短暂性脑缺血发作），或提示脑缺血的复发发作（一过性黑矇、复发性严重偏头痛）、网状青斑、超声心动图检查发现的心内膜瓣膜病变（11例二尖瓣，2例主动脉瓣），这些病变常与散在的赘生物相关、视网膜血管病变，以及计算机断层扫描/磁共振成像扫描或血管造影显示的多发性脑梗死证据。静脉血栓栓塞事件不常见（14例中有3例）。常见的实验室检查包括血小板减少（14例中有10例）、直接抗人球蛋白试验结果阳性（14例中有11例）和补体减少（14例中有11例）。在最初使用水杨酸盐或抗凝治疗（华法林）长达10年的随访中发现，虽然许多患者有提示脑缺血的复发症状，但主要的中风综合征未复发，也未出现新的发作。
多发性脑缺血病变和心内膜病变（包括瓣膜赘生物）的组合表明，这些脑缺血事件代表脑栓塞，并且这些脑栓塞事件起源于二尖瓣（较少见的是主动脉瓣）上的赘生物病变，并与狼疮抗凝物有关。
