Erkan D, Bateman H, Lockshin M D
Division of Rheumatology, Hospital for Special Surgery, Weill Medical College of Cornell University, New York, NY 10021, USA.
Lupus. 1999;8(7):560-4. doi: 10.1191/096120399678840846.
We describe two patients whose initial presentation of systemic lupus erythematosus (SLE) was accompanied by haemorrhagic episodes and significant coagulopathy. Further investigation demonstrated positive lupus anticoagulant and decreased Factor II (prothrombin) activity. Both patients were diagnosed with lupus anticoagulant-hypoprothrombinemia syndrome (LAC-HPS) as a result of non-neutralizing antibodies directed against Factor II. LAC-HPS is a rare clinical entity that can occur in association with SLE, transient viral infections, drug reactions or even in healthy individuals. Mixing studies, which can be affected by other coagulation factor inhibitors, play an important role in the diagnosis of LAC-HPS. Factor VII level was decreased in the second patient, a finding that has not previously been reported in association with SLE. In both patients, bleeding stopped promptly and coagulation studies improved significantly with high dose corticosteroids. We discuss the pathogenesis, diagnosis and management of LAC-HPS in patients with SLE.
我们描述了两名系统性红斑狼疮（SLE）初发时伴有出血发作和明显凝血病的患者。进一步检查显示狼疮抗凝物阳性且凝血因子II（凝血酶原）活性降低。两名患者均因针对凝血因子II的非中和抗体而被诊断为狼疮抗凝物-低凝血酶原血症综合征（LAC-HPS）。LAC-HPS是一种罕见的临床病症，可与SLE、短暂性病毒感染、药物反应甚至健康个体相关。混合试验可能会受到其他凝血因子抑制剂的影响，在LAC-HPS的诊断中起重要作用。第二名患者的凝血因子VII水平降低，这一发现此前尚未见与SLE相关的报道。两名患者的出血均迅速停止，高剂量皮质类固醇治疗后凝血研究显著改善。我们讨论了SLE患者中LAC-HPS的发病机制、诊断和管理。
