Considerations for simultaneous detection of autoantibodies to coagulation factor and lupus anticoagulant

M. Ieko, K. Ohmura, S. Naito, Mika Yoshida, Hisaomi Sasaki, Tsuyoshi Sato, Norifumi Sugawara, N. Takahashi, A. Ichinose
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Abstract

In patients with autoimmune coagulation factor deficiency (AiCFD), the production of autoantibodies that inhibit coagulation factors in the blood reduces the activity of those relevant coagulation factors, resulting in severe bleeding symptoms. Recently, reports of patients with AiCFD have noted the concomitant detection of lupus anticoagulant (LA), a risk factor for thrombosis. LA-positive patients may show bleeding symptoms due to decreased activity of coagulation factor II (FII) caused by autoantibodies against FII, in addition to thrombotic symptoms, a condition termed LA-hypoprothrombinemia syndrome (LAHPS). Anti-FII antibodies in LAHPS cases are frequently cleared antibodies that can be detected using immunological techniques, such as enzyme-linked immunosorbent assay (ELISA). Recently, several cases of coagulation FV inhibitors, known as autoimmune FV deficiency, have been reported. Some of these cases may be complicated by LA, which can cause thrombosis. False-positive results for anticoagulant inhibitors are known to occur in LA cases; therefore, immunological confirmation of antibodies against coagulation factors is recommended. Additionally, acquired hemophilia A (AHA), caused by autoantibodies against FVIII, is a typical acquired hemorrhagic diathesis, although affected patients may present with thrombosis associated with LA. Thus, it is important to remember that hemorrhagic diathesis due to autoantibodies against clotting factors can also result in thrombosis, as demonstrated by the co-detection of LA. When clotting factor inhibitors are detected in LA-positive individuals, it is important to confirm the presence of autoantibodies against coagulation factors using immunological methods, such as ELISA, to avoid false-positive results.
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凝血因子和狼疮抗凝剂自身抗体同时检测的考虑
在自身免疫性凝血因子缺乏症(AiCFD)患者中,血液中抑制凝血因子的自身抗体的产生会降低这些相关凝血因子的活性,从而导致严重的出血症状。最近,关于AiCFD患者的报告指出,同时检测到狼疮抗凝剂(LA),这是血栓形成的一个危险因素。LA阳性患者可能表现出出血症状,这是由于抗FII自身抗体引起的凝血因子II(FII)活性降低,此外还有血栓症状,这种情况被称为LA低凝血酶原综合征(LAHPS)。LAHPS病例中的抗FII抗体是经常被清除的抗体,可以使用免疫技术检测,如酶联免疫吸附试验(ELISA)。最近,已经报道了几种凝血FV抑制剂的病例,称为自身免疫性FV缺乏症。其中一些病例可能因左心房而变得复杂,从而导致血栓形成。已知抗凝抑制剂的假阳性结果发生在LA病例中;因此,建议对凝血因子抗体进行免疫学确认。此外,由抗FVIII自身抗体引起的获得性血友病A(AHA)是一种典型的获得性出血素质,尽管受影响的患者可能会出现与LA相关的血栓形成。因此,重要的是要记住,抗凝血因子自身抗体导致的出血素质也会导致血栓形成,正如LA的共同检测所证明的那样。当在LA阳性个体中检测到凝血因子抑制剂时,重要的是使用免疫方法(如ELISA)确认抗凝血因子自身抗体的存在,以避免假阳性结果。
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