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Alan Nurden: Platelet Defects Explain Bleeding in EHDS Patients
Alan Nurden/Photo: issue.com
Feb 25, 2026, 16:47

Alan Nurden: Platelet Defects Explain Bleeding in EHDS Patients

Alan Nurden, Emeritus Research Director at CNRS, Co-Founder of the French National Reference Centre for Inherited Platelet Disorders (CRPP), shared a post on LinkedIn about a recent article by Mariia Kumskova et al, published in Blood:

“While the Ehlers-Danlos syndrome (EHDS) is basically defined as a connective tissue disorder linked to increased elasticity many patients also report easy bruising and hemorrhagic problems. In this online paper in Blood, Mariia Kumskova and her colleagues describe how the platelet defects in EHDS patients are caused by decreased αIIbβ3 activation due to abnormally low talin-1 phosphorylation and a decreased presence of GPVI and PAR1, receptors for collagen and thrombin respectively.

As these signaling abnormalities were reproduced in a mouse (Col5a1+/-) model, it is therefore probable that the platelet defects directly explain the bleeding confirming that EHDS patients also have an inherited platelet disorder.

Interestingly, just as in Glanzmann thrombasthenia (where alphaIIbbeta3 function is absent), previous studies have shown that while thrombotic risk is reduced for EHDS patients, rare cases still occur and the general cardiovascular burden is not mitigated. An original and impressive study.”

Title: Platelet defects in patients and mice with Ehlers-Danlos syndrome

Authors: Mariia Kumskova, Gagan D Flora, Manasa K. Nayak, Ivan Budnik, Aditi Jain, Rakesh B. Patel, Abhishek B Jha, Madankumar Ghatge, Neelam Chauhan, James V. Michael, Steven E. McKenzie, Anjali Sharathkumar, Janice M Staber, Steven R Lentz, Anil Chauhan

Read the Full Article on Blood.

Alan Nurden

Other articles featuring Alan Nurden on Hemostasis Today.

Abhishek B Jha Aditi Jain Alan Nurden Anil Chauhan Anjali Sharathkumar Blood Collagen EHDS Ehlers-Danlos Syndromes Ehlers–Danlos Syndrome Gagan D Flora Glanzmann thrombasthenia Health Hemostasis Today Inherited Platelet Disorder Ivan Budnik James V. Michael Janice M. Staber Madankumar Ghatge Manasa K. Nayak Mariia Kumskova Medicine Neelam Chauhan Platelet Platelet Defects Rakesh B. Patel Steven E. McKenzie Steven R. Lentz
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