Department of Internal Medicine, University of Iowa, Iowa City, Iowa, USA.
Department of Internal Medicine, University of Iowa, Iowa City, Iowa, USA.
J Thromb Haemost. 2023 Jul;21(7):1824-1830. doi: 10.1016/j.jtha.2023.04.004. Epub 2023 Apr 17.
Easy bruising is included as a major or minor criterion for the classification of multiple types of Ehlers-Danlos syndrome (EDS). Despite a longstanding recognition of the association between EDS and bleeding, we still lack a definitive understanding of the frequency, severity, and types of bleeding complications in patients with EDS.
To evaluate hemorrhagic symptoms using the International Society of Thrombosis and Haemostasis bleeding assessment tool (ISTH-BAT) in a cohort of patients with defined types of EDS.
We utilized the ISTH-BAT to characterize hemorrhagic symptoms and their severity in a cohort of 52 patients with classical, classical-like, hypermobile, or vascular EDS and a matched group of 52 healthy control subjects.
The mean ISTH-BAT score was 0.1 for healthy subjects and 9.1 for patients with EDS (p < .0001). An abnormal ISTH-BAT score was observed in 32 of 52 (62%) patients with EDS and 0 of 52 healthy controls (p < .0001). The most frequent bleeding symptoms were bruising, muscle hematomas, menorrhagia, epistaxis, bleeding from the oral cavity, and bleeding after tooth extraction. Menorrhagia that was life-threatening or required surgery was reported in 7 of 52 (14%) patients with EDS.
Patients with multiple types of EDS exhibit a wide range of bleeding symptoms ranging from mild to life-threatening episodes.
容易瘀伤被包括在多种类型的埃勒斯-当洛斯综合征（EDS）的主要或次要标准中。尽管人们早就认识到 EDS 与出血之间存在关联，但我们仍然缺乏对 EDS 患者出血并发症的频率、严重程度和类型的明确理解。
使用国际血栓与止血学会出血评估工具（ISTH-BAT）评估特定类型 EDS 患者的出血症状。
我们使用 ISTH-BAT 对 52 名经典型、经典型样、高活动型或血管型 EDS 患者和 52 名匹配的健康对照者的出血症状及其严重程度进行了特征描述。
健康对照组的平均 ISTH-BAT 评分为 0.1，EDS 患者为 9.1（p<0.0001）。32 名 EDS 患者（62%）的 ISTH-BAT 评分异常，而 52 名健康对照组均无异常（p<0.0001）。最常见的出血症状是瘀伤、肌肉血肿、月经过多、鼻出血、口腔出血和拔牙后出血。7 名 EDS 患者（14%）报告月经过多危及生命或需要手术。
多种类型的 EDS 患者表现出从轻度到危及生命的各种出血症状。
