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.
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