Department of Haematology, Erasmus University Medical Centre, Rotterdam, The Netherlands.
Department of Paediatric Haematology, Erasmus University Medical Centre-Sophia Children's Hospital, Rotterdam, The Netherlands.
Thromb Haemost. 2021 Nov;121(11):1409-1416. doi: 10.1055/s-0041-1726344. Epub 2021 Apr 14.
The most optimal management for patients with bleeding of unknown cause (BUC) is unknown, as limited data are available.
Evaluate management and outcome of surgical procedures and deliveries in patients with BUC.
All patients ≥12 years of age, referred to a tertiary center for a bleeding tendency, were included. Bleeding phenotype was assessed and hemostatic laboratory work-up was performed. Patients were diagnosed with BUC or an established bleeding disorder (BD). Data on bleeding and treatment during surgical procedures and delivery following diagnosis were collected.
Of 380 included patients, 228 (60%) were diagnosed with BUC and 152 (40%) with an established BD. In 14/72 (19%) surgical procedures major bleeding occurred and 14/41 (34%) deliveries were complicated by major postpartum hemorrhage (PPH). More specifically, 29/53 (55%) of the BUC patients who underwent surgery received prophylactic treatment to support hemostasis. Despite these precautions, 4/29 (14%) experienced major bleeding. Of BUC patients not treated prophylactically, bleeding occurred in 6/24 (25%). Of pregnant women with BUC, 2/26 (8%) received prophylactic treatment during delivery, one women with and 11 (46%) women without treatment developed major PPH.
Bleeding complications are frequent in BUC patients, irrespective of pre- or perioperative hemostatic treatment. We recommend a low-threshold approach toward administration of hemostatic treatment in BUC patients, especially during delivery.
由于可用数据有限,对于不明原因出血(BUC)患者,最佳治疗方法尚不清楚。
评估 BUC 患者手术和分娩的治疗方法和结局。
所有年龄≥12 岁、因出血倾向被转诊至三级中心的患者均被纳入本研究。评估出血表型并进行止血实验室检查。将患者诊断为 BUC 或已确诊的出血性疾病(BD)。收集确诊后手术和分娩期间的出血和治疗数据。
在纳入的 380 例患者中,228 例(60%)被诊断为 BUC,152 例(40%)为已确诊的 BD。72 例手术中有 14 例(19%)发生大出血,41 例分娩中有 14 例(34%)发生严重产后出血(PPH)。更具体地说,53 例接受手术的 BUC 患者中有 29 例接受了预防性止血治疗。尽管采取了这些预防措施,但仍有 4/29(14%)例患者发生大出血。未预防性治疗的 BUC 患者中,有 6/24(25%)例患者发生出血。26 例 BUC 孕妇中有 2 例(8%)在分娩时接受了预防性治疗,1 例(46%)接受治疗的孕妇和 11 例(46%)未接受治疗的孕妇发生了严重 PPH。
无论是否进行围手术期止血治疗,BUC 患者的出血并发症都很常见。我们建议对 BUC 患者,尤其是在分娩期间,采用低门槛的止血治疗方法。