Cohen H, Mackie I J, Anagnostopoulos N, Savage G F, Machin S J
Department of Haematology, University College and Middlesex School of Medicine, London.
J Clin Pathol. 1989 Jun;42(6):629-33. doi: 10.1136/jcp.42.6.629.
The prevalence of lupus anticoagulant, using the dilute Russell's viper venom time (DRVT), was determined in 22 patients with mild to severe haemophilia A to see if there was any association with the presence of viral disease. Twelve haemophiliacs (58%) were lupus anticoagulant positive, with a mean patient:control ratio of 1.24 (range 1.15-1.52, normal range 0.84-1.06 which partially corrected with lysed, washed platelets). Nine of these patients were IgG or IgM, or both, anticardiolipin antibody positive and nine were human immunodeficiency virus (HIV) antibody positive, but associations between lupus anticoagulant, anticardiolipin antibodies, and HIV antibody positivity were not significant. Mixing studies of normal plasma and immune depleted factor VIII deficient plasma showed that the DRVT ratio increased when the factor VIII concentration fell below 0.15 IU/ml. There was no significant association between plasma factor VIII concentration and positive DRVT results in haemophiliacs. The addition of porcine factor VIII concentrate produced no correction in eight of the 12 with DRVTs indicative of lupus anticoagulant, suggesting that these were prolonged by antiphospholipid activity. It is concluded that the presence of lupus anticoagulant and anticardiolipin antibodies in haemophiliacs may represent an antiphospholipid response to viraemic challenge, not only to HIV but also to other viral antigens, and that a very low factor VIII concentration may produce a false positive DRVT result.
采用稀释蝰蛇毒时间(DRVT)测定了22例轻至重度甲型血友病患者狼疮抗凝物的患病率,以观察其与病毒病存在与否的相关性。12例血友病患者(58%)狼疮抗凝物阳性,患者与对照的平均比值为1.24(范围1.15 - 1.52,正常范围0.84 - 1.06,用溶解、洗涤的血小板可部分纠正)。其中9例患者IgG或IgM或两者抗心磷脂抗体阳性,9例人类免疫缺陷病毒(HIV)抗体阳性,但狼疮抗凝物、抗心磷脂抗体和HIV抗体阳性之间的相关性不显著。正常血浆与免疫耗竭的因子VIII缺乏血浆的混合研究表明,当因子VIII浓度降至0.15 IU/ml以下时,DRVT比值升高。血友病患者血浆因子VIII浓度与DRVT阳性结果之间无显著相关性。在12例DRVT提示狼疮抗凝物的患者中,8例加入猪因子VIII浓缩物后未得到纠正,提示这些结果是由抗磷脂活性延长的。结论是,血友病患者中狼疮抗凝物和抗心磷脂抗体的存在可能代表对病毒血症挑战的抗磷脂反应,不仅针对HIV,也针对其他病毒抗原,而且极低的因子VIII浓度可能产生假阳性的DRVT结果。