Pathophysiological insights in sickle cell disease.
Article
in English
| IMSEAR
| ID: sea-136354
ABSTRACT
The first coherent pathophysiological scheme for sickle cell disease (SCD) emerged in the sixties-seventies based on an extremely detailed description of the molecular mechanism by which HbS in its deoxy-form polymerises and forms long fibres within the red blood cell that deform it and make it fragile. This scheme explains the haemolytic anaemia, and the mechanistic aspects of the vaso-occlusive crises (VOCs), but, even though it constitutes the basic mechanism of the disease, it does not account for the processes that actually trigger VOCs. This paper reviews recent data which imply red blood cell dehydration, its abnormal adhesion properties to the endothelium, the participation of inflammatory phenomenon and of a global activation of all the cells present in the vessel, and finally, abnormalities of the vascular tone and of nitric oxide metabolism. These data altogether have shed a new light on the pathophysiology of the first molecular disease i.e. sickle cell disease.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Main subject:
Humans
/
Hemoglobin, Sickle
/
Endothelium, Vascular
/
Cell Adhesion
/
Erythrocytes
/
Hemolysis
/
Ion Channels
/
Anemia, Sickle Cell
/
Nitric Oxide
Language:
English
Year:
2011
Type:
Article
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