Characterization of bleeding symptoms in Ehlers–Danlos syndrome, 2023, Kumskova et al.

Characterization of bleeding symptoms in Ehlers–Danlos syndrome
Mariia Kumskova; Gagan D. Flora; Janice Staber; Steven R. Lentz; Anil K. Chauhan

Background
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.

Objectives
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.

Methods
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.

Results
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.

Conclusion
Patients with multiple types of EDS exhibit a wide range of bleeding symptoms ranging from mild to life-threatening episodes.


Link | PDF (Journal of Thrombosis and Haemostasis)

 

This one is not open access but is discussed in this recent article: Bleeding Symptoms in Patients With Ehlers-Danlos Syndrome Range From Mild to Life-Threatening