Blood rheological abnormalities in sickle cell anemia.

Abstract:

:This review focuses on the contribution of abnormal blood rheology in the pathophysiology of sickle cell anemia (SCA). SCA is characterized by a reduction of red blood cell (RBC) deformability but this reduction is very heterogeneous among patients. Recent works have shown that patients with the lowest RBC deformability (measured by ektacytometry) have enhanced hemolysis and would be more prone to develop several complications such as priapism, leg ulcers and glomerulopathy. In contrast, patients with the highest deformability, and not under hydroxyurea therapy, seem to develop more frequently vaso-occlusive like events. Although less studied, RBC aggregation properties are very different between SCA and healthy individuals and it was demonstrated that increased RBC aggregates strength could be involved in some complications. Finally, several studies have established that the vascular system of SCA patients could not fully compensate any increase in blood viscosity because of the loss of vascular reactivity, which may result in vaso-occlusive crises.

authors

Connes P,Renoux C,Romana M,Abkarian M,Joly P,Martin C,Hardy-Dessources MD,Ballas SK

doi

10.3233/CH-189005

subject

Has Abstract

pub_date

2018-01-01 00:00:00

pages

165-172

issue

2-3

eissn

1386-0291

issn

1875-8622

pii

CH189005

journal_volume

68

pub_type

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