Manco-Johnson M J
Department of Pediatrics, University of Colorado Health Sciences Center and The Children's Hospital, Denver, USA.
Semin Thromb Hemost. 1998;24(6):591-8. doi: 10.1055/s-2007-996059.
Lupus anticoagulant (LA) is made up of heterogeneous IgG and IgM antibodies that prolong clotting times in vitro and is associated with an increased rate of both thrombosis and hemorrhage in vivo, although thrombosis is far more common. Many mechanisms of action have been explored, but none explains the coagulation abnormality of every sample tested. Binding of these antibodies to protein phospholipid complexes provides a unifying model. Antiphospholipid antibodies (APAs) are found in adult patients with a variety of disorders or as an isolated finding. The association of LA and anticardiolipin antibodies (ACAs) with thrombosis in adults has been established, although there is no test as yet to predict thrombotic risk for an asymptomatic affected individual. The presentation of thrombosis with postinfectious APA is uncommon in adults. Children who present with thrombosis and LA are found to have underlying disorders similar to those of adults. Although the presentation of thrombosis in children with postinfectious LA is rare, the association is established. LA-positive children with thrombosis have manifested a severe acquired deficiency of protein S; LA-positive children with hemorrhage have manifested an acquired deficiency of prothrombin. The association of thrombosis with ACA-positive children has been reported. Further work to determine the epidemiology, mechanism of action, and thrombotic potential of APA in children is warranted to better understand, prevent, and treat thrombotic and hemorrhagic complications.
狼疮抗凝物（LA）由异质性IgG和IgM抗体组成，可在体外延长凝血时间，并且在体内与血栓形成和出血发生率增加相关，尽管血栓形成更为常见。人们已经探索了许多作用机制，但没有一种能解释所检测的每个样本的凝血异常情况。这些抗体与蛋白质磷脂复合物的结合提供了一个统一的模型。抗磷脂抗体（APA）见于患有多种疾病的成年患者，或作为一项单独发现。LA和抗心磷脂抗体（ACA）与成人血栓形成之间的关联已经确立，尽管目前尚无检测方法可预测无症状受累个体的血栓形成风险。感染后APA所致血栓形成在成人中并不常见。出现血栓形成和LA的儿童被发现有与成人相似的潜在疾病。尽管感染后LA患儿出现血栓形成的情况很少见，但这种关联已得到证实。LA阳性的血栓形成患儿表现出严重的获得性蛋白S缺乏；LA阳性的出血患儿表现出获得性凝血酶原缺乏。ACA阳性儿童与血栓形成之间的关联已有报道。有必要进一步开展工作，以确定儿童APA的流行病学、作用机制和血栓形成潜能，从而更好地理解、预防和治疗血栓形成及出血并发症。
