Haematologica, Vol 93, Issue 2, 307-308 doi:10.3324/haematol.12027
Copyright © 2008 by Ferrata Storti Foundation
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Sickle Cell Disease

Sickle cell patients are characterized by a reduced glycocalyx volume

Eduard J. van Beers1, Max Nieuwdorp2, Ashley J. Duits3, Ludo M. Evers1, John-John B. Schnog4, Bart J. Biemond1, on behalf of the CURAMA study group

1 Department of Hematology, Academic Medical Centre, Amsterdam, The Netherlands;
2 Department of Vascular Medicine, Academic Medical Centre, Amsterdam, The Netherlands;
3 Immunology Department, Red Cross Blood Bank Foundation, Curaçao, Netherlands Antilles;
4 Department of Hematology, Erasmus Medical Centre, Rotterdam, The Netherlands

Correspondence: Bart J. Biemond, MD, PhD, Department of Haematology, F4-224, Academic Medical Centre, PO box 22660 1100 DD Amsterdam, The Netherlands. Phone: international +31.20.5665785. Fax: international +31.20.6919743 E-mail: b.j.biemond{at}amc.uva.nl

The glycocalyx is an important anti-inflammatory and anti-adhesive barrier at the luminal side of endothelial cells. Glycocalyx volume was significantly reduced in sickle cell patients (HbSS/HbSβ0–tha-lassemia median 0.47L, IQR 0.27–0.66, HbSC/HbSβ+-thalassemia 0.23L, 0.0–0.58) compared with controls (1x109L, 0.52–1.77) (p=0.03). Reduced glycocalyx may be a new factor in the pathophysiology of sickle cell disease.




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