Department of Haematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands.
Department of Paediatric Haematology, Erasmus University Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands.
Haemophilia. 2020 May;26(3):e106-e115. doi: 10.1111/hae.13991. Epub 2020 Apr 26.
Diagnostic evaluation of patients with a bleeding tendency remains challenging, as no disorder is identified in approximately 50% of patients. An impaired interplay of several haemostatic factors might explain bleeding phenotype in these patients.
To investigate whether global haemostasis assays are able to identify haemostatic abnormalities in patients with a bleeding tendency unexplained by current diagnostic laboratory tests.
Patients of ≥12 years with a bleeding tendency were included from a tertiary outpatient clinic. Bleeding phenotype was assessed with the ISTH-BAT. Patients were classified as having bleeding of unknown cause (BUC) or a mild bleeding disorder (MBD) based on abnormalities assessed by routine haemostatic tests. Global haemostasis tests (rotational thromboelastometry (ROTEM), thrombin generation test (TG) and plasma clot lysis time (CLT)) were measured in all patients. The results were compared with 76 controls.
One hundred and eighty-one patients were included, and 60% (109/181) was classified as having BUC. BUC patients demonstrated a significantly prolonged lag time in TG (median 7.7 minutes, IQR 6.7-8.7) and a significantly prolonged CLT (median 60.5 minutes, IQR 54.7-66.1) compared to controls. No differences in ROTEM variables were found. Patients with MBD showed an impaired thrombin generation with a significantly decreased ETP (median 1024 nmol/L*min, IQR 776-1355) and peak height (median 95 nmol/L, IQR 76-138), compared to BUC patients and controls.
No major differences were found in ROTEM and TG variables in BUC patients compared to controls. BUC patients did have a significantly prolonged clot lysis time. The underlying mechanism for this finding is unknown.
对于有出血倾向的患者，其诊断评估仍然具有挑战性，因为大约 50%的患者未发现任何疾病。几种止血因子相互作用的障碍可能解释了这些患者的出血表型。
研究是否可以通过全局止血检测来识别目前诊断性实验室检测无法解释的有出血倾向患者的止血异常。
从三级门诊诊所招募了年龄≥12 岁的有出血倾向的患者。使用 ISTH-BAT 评估出血表型。根据常规止血检测评估的异常情况，将患者分为出血原因不明（BUC）或轻度出血性疾病（MBD）。所有患者均测量全局止血检测（旋转血栓弹性测定法（ROTEM）、凝血酶生成试验（TG）和血浆凝块溶解时间（CLT））。将结果与 76 名对照进行比较。
共纳入 181 例患者，其中 60%（109/181）被归类为 BUC。与对照组相比，BUC 患者的 TG 潜伏期明显延长（中位数 7.7 分钟，IQR 6.7-8.7），CLT 明显延长（中位数 60.5 分钟，IQR 54.7-66.1）。ROTEM 变量无差异。MBD 患者的凝血酶生成受损，ETP 明显降低（中位数 1024 nmol/L*min，IQR 776-1355），峰高明显降低（中位数 95 nmol/L，IQR 76-138），与 BUC 患者和对照组相比。
与对照组相比，BUC 患者的 ROTEM 和 TG 变量无明显差异。BUC 患者确实有明显延长的凝块溶解时间。这种发现的潜在机制尚不清楚。
