Haemophilia Centre, University Hospital of Wales, Cardiff, UK.
Department of Haematology, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
J Thromb Haemost. 2022 Nov;20(11):2519-2525. doi: 10.1111/jth.15871. Epub 2022 Sep 21.
Patients with a bleeding tendency with normal laboratory tests have been described as having an unclassified bleeding disorder or bleeding disorder of unknown cause (BDUC). There are very little data available on how to manage pregnancy.
To study management and outcomes of these patients at four United Kingdom hemophilia comprehensive care centers.
Retrospective case note review from 2010-2020.
Sixty deliveries in 36 patients were recorded. The median International Society on Thrombosis and Haemostasis bleeding assessment tool score was 9. In 54 cases for which data were available, the odds ratio for post partum hemorrhage (PPH) was 6.3 for no primary hemostatic prophylaxis versus prophylaxis (95% confidence interval 1.2-34.2, p < .05); 7/9 (78%) versus 16/45 (36%) PPH incidence for the groups, respectively. Hemostatic prophylaxis was with tranexamic acid but some patients received desmopressin or platelet infusions. Secondary PPH was seen in 5/60 (8%) of cases. No neonatal bleeding complications or maternal thromboembolic complications were noted. Avoidance of regional anesthesia and fetal delivery precautions were commonly advised, but in the small number of cases in which they occurred no complications were noted.
Despite hemostatic prophylaxis PPH was commonly seen. Further prospective studies of BDUC patients are required to determine optimal management in pregnancy as well as determine the pathophysiological basis of bleeding.
实验室检查正常但存在出血倾向的患者被描述为患有未分类出血性疾病或不明原因出血性疾病（BDUC）。关于如何管理妊娠，数据非常有限。
研究英国四家血友病综合护理中心的这些患者的管理和结局。
回顾性病例记录审查，时间为 2010 年至 2020 年。
记录了 36 名患者中的 60 次分娩。国际血栓与止血学会出血评估工具评分中位数为 9 分。在 54 例可获得数据的情况下，无原发性止血预防与预防相比（95%置信区间为 1.2-34.2，p<0.05），产后出血（PPH）的比值比为 6.3；分别为两组的 7/9（78%）和 16/45（36%）的 PPH 发生率。止血预防使用氨甲环酸，但部分患者接受了去氨加压素或血小板输注。5/60（8%）的病例出现继发性 PPH。未观察到新生儿出血并发症或母体血栓栓塞并发症。通常建议避免区域麻醉和胎儿分娩预防措施，但在发生这些并发症的少数情况下，未观察到并发症。
尽管进行了止血预防，但仍常见 PPH。需要对 BDUC 患者进行进一步的前瞻性研究，以确定妊娠期间的最佳管理方法，并确定出血的病理生理学基础。
