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Purpose <p>Ehlers–Danlos syndromes (EDS) are a group of connective tissue disorders associated with an increased risk of bleeding. We examined the association between point-of-care (POC) testing parameters with bleeding history in a cohort cared for at the GoodHope EDS Clinic at Toronto General Hospital.</p> Methods <p>We conducted an exploratory cross-sectional study at the Toronto General Hospital GoodHope EDS Clinic, recruiting adult patients with a diagnosis of EDS from 27 April 2022 to 7 October 2024. Patients provided samples for rotational thromboelastometry (ROTEM) and functional platelet testing (PlateletWorks), and were administered the International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH-BAT) to calculate the Bleeding Severity Score (BSS). The association between the BSS and POC parameters was examined using descriptive statistics and Spearman correlation analysis.</p> Results <p>We recruited 17 patients (hypermobile subtype, <i>n</i> = 10; classical subtype, <i>n</i> = 7). Clinically significant bleeding (BSS ≥ 4 in males; ≥ 6 in females) was reported in the majority (classical group, 86%; hypermobile group, 90%). Nine (53%) patients had major surgery or trauma at a median age of 23 (range, 8–39) yr. There were no significant abnormalities detected using ROTEM. Lower functional platelet percentages were observed in the hypermobile subgroup, with no association with BSS.</p> Conclusions <p>Point-of-care functional platelet testing using aggregation in response to an exogenous collagen agonist may detect inherent platelet dysfunction in patients with hypermobile subtype. We did not detect any ROTEM parameter abnormalities that were associated with elevated BSS scores. This supports a multifactorial etiology of bleeding in this patient population.</p>

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The association of point-of-care coagulation testing with bleeding symptoms in patients with Ehlers–Danlos syndrome: an exploratory cross-sectional study

  • 期刊论文

  • 发表日期：

    2025年11月1日

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  • Austin L. Lam,
  • Nimish Mittal,
  • Michelle Vinod,
  • Keyvan Karkouti,
  • Hance Clarke,
  • Justyna Bartoszko

摘要

Purpose

Ehlers–Danlos syndromes (EDS) are a group of connective tissue disorders associated with an increased risk of bleeding. We examined the association between point-of-care (POC) testing parameters with bleeding history in a cohort cared for at the GoodHope EDS Clinic at Toronto General Hospital.

Methods

We conducted an exploratory cross-sectional study at the Toronto General Hospital GoodHope EDS Clinic, recruiting adult patients with a diagnosis of EDS from 27 April 2022 to 7 October 2024. Patients provided samples for rotational thromboelastometry (ROTEM) and functional platelet testing (PlateletWorks), and were administered the International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH-BAT) to calculate the Bleeding Severity Score (BSS). The association between the BSS and POC parameters was examined using descriptive statistics and Spearman correlation analysis.

Results

We recruited 17 patients (hypermobile subtype, n = 10; classical subtype, n = 7). Clinically significant bleeding (BSS ≥ 4 in males; ≥ 6 in females) was reported in the majority (classical group, 86%; hypermobile group, 90%). Nine (53%) patients had major surgery or trauma at a median age of 23 (range, 8–39) yr. There were no significant abnormalities detected using ROTEM. Lower functional platelet percentages were observed in the hypermobile subgroup, with no association with BSS.

Conclusions

Point-of-care functional platelet testing using aggregation in response to an exogenous collagen agonist may detect inherent platelet dysfunction in patients with hypermobile subtype. We did not detect any ROTEM parameter abnormalities that were associated with elevated BSS scores. This supports a multifactorial etiology of bleeding in this patient population.