Abstract：
BACKGROUND: Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare disease caused by acquired factor II (FII) deficiency and lupus anticoagulant. Patients with LAHPS typically present with thrombosis and bleeding. However, little information is available on the evaluation of coagulation potential in patients with LAHPS. We examined global coagulation potentials in patients with LAHPS during the clinical course in this study.
METHODS: Coagulation potentials in two pediatric patients with LAHPS were assessed by measuring clotting time (CT) and clot formation time using Ca2+-triggered rotational thromboelastometry (ROTEM), CT and maximum coagulation velocity using clot waveform analysis (CWA), and lag time and peak thrombin using the thrombin generation assay (TGA). The day of admission was defined as day 0.
RESULTS: In case 1, the bleeding symptoms disappeared by day 5. However, the TGA and CWA results were markedly lower than normal, although FII activity (FII:C) returned to within the normal range by day 14. In contrast, ROTEM revealed a recovery to near-normal levels (day 14). All coagulation parameters (day 80) were within normal ranges. In case 2, coagulation potential was severely depressed until day 12, although FII:C returned to normal levels. Bleeding symptoms disappeared on day 19, and the ROTEM data revealed that the parameters were close to the normal range. The coagulation parameters in all assays were normalized on day 75.
CONCLUSIONS: Recovery of coagulation potential in patients with LAHPS was slower than the recovery of FII:C. Moreover, ROTEM appeared to be clinically useful for assessing coagulation potential in patients with LAHPS.
METHODS: Coagulation potentials in two pediatric patients with LAHPS were assessed by measuring clotting time (CT) and clot formation time using Ca2+-triggered rotational thromboelastometry (ROTEM), CT and maximum coagulation velocity using clot waveform analysis (CWA), and lag time and peak thrombin using the thrombin generation assay (TGA). The day of admission was defined as day 0.
RESULTS: In case 1, the bleeding symptoms disappeared by day 5. However, the TGA and CWA results were markedly lower than normal, although FII activity (FII:C) returned to within the normal range by day 14. In contrast, ROTEM revealed a recovery to near-normal levels (day 14). All coagulation parameters (day 80) were within normal ranges. In case 2, coagulation potential was severely depressed until day 12, although FII:C returned to normal levels. Bleeding symptoms disappeared on day 19, and the ROTEM data revealed that the parameters were close to the normal range. The coagulation parameters in all assays were normalized on day 75.
CONCLUSIONS: Recovery of coagulation potential in patients with LAHPS was slower than the recovery of FII:C. Moreover, ROTEM appeared to be clinically useful for assessing coagulation potential in patients with LAHPS.
摘要：
背景：狼疮抗凝-低凝血酶原血症综合征（LAHPS）是一种由获得性II因子（FII）缺乏和狼疮抗凝药引起的罕见疾病。患有LAHPS的患者通常表现为血栓形成和出血。然而,关于LAHPS患者凝血电位评估的信息很少.在这项研究的临床过程中，我们检查了LAHPS患者的整体凝血电位。
方法：通过使用Ca2+触发的旋转血栓弹性测定法（ROTEM）测量凝血时间（CT）和凝块形成时间来评估两名患有LAHPS的儿科患者的凝血电位，使用凝块波形分析（CWA）的CT和最大凝血速度，以及使用凝血酶生成测定(TGA)的滞后时间和峰值凝血酶。入院日定义为第0天。
结果：在病例1中，出血症状在第5天消失。然而,TGA和CWA结果明显低于正常，尽管FII活性(FII:C)在第14天恢复到正常范围内。相比之下,ROTEM显示恢复至接近正常水平(第14天)。所有凝血参数(第80天)均在正常范围内。在病例2中,尽管FII:C恢复到正常水平,但凝血潜能严重降低直到第12天。出血症状在第19天消失，ROTEM数据显示参数接近正常范围。在第75天对所有测定中的凝血参数进行归一化。
结论：LAHPS患者的凝血电位恢复比FII:C的恢复慢。此外，ROTEM似乎在临床上可用于评估LAHPS患者的凝血潜能。
方法：通过使用Ca2+触发的旋转血栓弹性测定法（ROTEM）测量凝血时间（CT）和凝块形成时间来评估两名患有LAHPS的儿科患者的凝血电位，使用凝块波形分析（CWA）的CT和最大凝血速度，以及使用凝血酶生成测定(TGA)的滞后时间和峰值凝血酶。入院日定义为第0天。
结果：在病例1中，出血症状在第5天消失。然而,TGA和CWA结果明显低于正常，尽管FII活性(FII:C)在第14天恢复到正常范围内。相比之下,ROTEM显示恢复至接近正常水平(第14天)。所有凝血参数(第80天)均在正常范围内。在病例2中,尽管FII:C恢复到正常水平,但凝血潜能严重降低直到第12天。出血症状在第19天消失，ROTEM数据显示参数接近正常范围。在第75天对所有测定中的凝血参数进行归一化。
结论：LAHPS患者的凝血电位恢复比FII:C的恢复慢。此外，ROTEM似乎在临床上可用于评估LAHPS患者的凝血潜能。
