Abstract:
There is no established method for differentiating acquired hemophilia A (AHA) from lupus anticoagulant (LA) positivity because both present with prolonged activated partial thromboplastin time. We compared various parameters of rotational thromboelastometry (ROTEM), thrombin generation assay (TGA), and clot waveform analysis (CWA) in patients with AHA (n = 10) and LA (n = 44). Compared with AHA, possible (n = 12) and definite (n = 32) LA showed significantly shorter clotting time (CT) in NATEM mode of ROTEM (> 3600 vs. 501/533). In TGA, peak height was significantly lower in AHA (16 vs. 242/174 nM). In CWA, CT was significantly longer (81 vs. 36/41 s) and Ad|min1| was lower (2.1 vs. 8.7/6.7) in AHA. Notably, CT by NATEM and peak height in TGA completely discriminated between AHA and LA, whereas Ad|min1| did not discriminate between them in 4 cases of AHA and 1 of LA. Comparison of 3 patients with both AHA and LA against a patient with only LA and markedly low FVIII activity (3.5%) showed that both CT by NATEM and peak height of TGA precisely classified the former 3 cases as AHA and the latter 1 case as LA, whereas Ad|min1| classified all 4 cases as AHA. ROTEM and TGA can comparably distinguish between AHA and LA.
摘要:
没有确定的方法可以区分获得性血友病A(AHA)和狼疮抗凝药(LA)阳性,因为两者都存在延长的活化部分凝血活酶时间。我们比较了旋转血栓弹性测量(ROTEM)的各种参数,凝血酶生成测定(TGA),AHA(n=10)和LA(n=44)患者的血块波形分析(CWA)。与AHA相比,可能(n=12)和明确(n=32)LA在ROTEM的NATEM模式下显示出明显较短的凝血时间(CT)(>3600vs.501/533)。在TGA,AHA的峰高明显较低(16vs.242/174nM)。在CWA,CT明显更长(81vs.36/41s)和Ad|min1|较低(2.1与8.7/6.7)在AHA中。值得注意的是,NATEM的CT和TGA的峰高完全区分AHA和LA,而Ad|min1|在4例AHA和1例LA中没有区分它们。3例同时患有AHA和LA的患者与仅患有LA且FVIII活性明显较低(3.5%)的患者的比较表明,NATEM的CT和TGA的峰高都将前3例精确分类为AHA,后1例精确分类为LA。而Ad|min1|将所有4例病例归类为AHA。ROTEM和TGA可以比较地区分AHA和LA。