Berkowitz Callie, Ma Alice, Miller Vanessa, Goraya Supreet, Kirkland Kristi, Grabell Julie, Key Nigel S, James Paula D
Division of Hematology and Blood Research Center, University of North Carolina, Chapel Hill, North Carolina, USA.
Department of Obstetrics and Gynecology, University of North Carolina, Chapel Hill, North Carolina, USA.
Res Pract Thromb Haemost. 2024 Sep 16;8(7):102572. doi: 10.1016/j.rpth.2024.102572. eCollection 2024 Oct.
Bleeding disorder of unknown cause (BDUC) is a diagnostic category encompassing patients with a clear bleeding phenotype but without identifiable abnormality on hemostatic testing. The optimal management of hemostasis in BDUC patients prior to invasive procedures and childbirth is uncertain.
Our objective was to characterize periprocedural hemostatic prophylaxis and bleeding outcomes in patients with BDUC.
We conducted a retrospective cohort study of adult patients with BDUC at 2 academic medical centers. Following diagnosis of BDUC, subsequent surgical procedures and childbirths were analyzed using a combination of registry data and manual chart review.
We identified 127 patients with mean age of 39.9 years (SD = 16.6); the majority of patients were female (91.3%). Forty-eight major procedures, 70 minor procedures, and 19 childbirths were analyzed. Antifibrinolytic monotherapy was advised for 57% of major procedures, 59% of minor procedures, and 67% of childbirths. Perioperative platelet transfusion was recommended in 26% of major procedures and 9% of minor procedures in combination with other hemostatic agents. Major or clinically relevant nonmajor bleeding occurred in 4.1% (4/98) of procedures with prophylaxis and 10% (2/20) of procedures without prophylaxis. Postpartum hemorrhage occurred in 26% (5/19) of deliveries.
In this multiinstitution experience, we found overall low rates of hemostatic complications in procedures completed with hemostatic prophylaxis, although preventing hemorrhage in childbirth and gynecologic procedures remain unmet needs.
不明原因出血性疾病（BDUC）是一个诊断类别，涵盖具有明确出血表型但止血检测未发现可识别异常的患者。BDUC患者在侵入性操作和分娩前的最佳止血管理尚不确定。
我们的目的是描述BDUC患者围手术期止血预防措施和出血结局。
我们对2个学术医疗中心的成年BDUC患者进行了一项回顾性队列研究。在诊断为BDUC后，使用登记数据和人工病历审查相结合的方式分析随后的外科手术和分娩情况。
我们确定了127例患者，平均年龄39.9岁（标准差=16.6）；大多数患者为女性（91.3%）。分析了48例大手术、70例小手术和19例分娩情况。57%的大手术、59%的小手术和67%的分娩建议采用抗纤溶单药治疗。26%的大手术和9%的小手术建议围手术期输注血小板，并联合使用其他止血剂。有预防措施的手术中，4.1%（4/98）发生了大出血或临床相关的非大出血，无预防措施的手术中这一比例为10%（2/20）。26%（5/19）的分娩发生了产后出血。
在这项多机构经验中，我们发现采用止血预防措施完成的手术中，止血并发症总体发生率较低，尽管在分娩和妇科手术中预防出血仍未满足需求。
