Fisiopatología de la vaso-oclusión en la drepanocitosis / Pathophysiology of the vaso-occlusion in the sickle cell anemia
Abstract
La vaso-oclusión en la drepanocitosis es una característica única entre las anemias hemolíticas. La idea de que el eritrocito falciforme induce el proceso vaso-oclusivo ha sido desechada y no cabe duda que el fenómeno ocurre debido a la adhesión de los hematíes deformables menos densos (reticulocitos de stress) al endotelio vascular activado en las vénulas post-capilares, proceso en el que participan moléculas de adhesión celular (MAC) eritrocitarias y vasculares así como un conjunto de factores plasmáticos; la externalización de la fosfatidilserina, la acción de la trombina, la expresión de factor tisular asociada a alteraciones del mecanismo de transporte catiónico, conjuntamente con la formación de agregados de banda 3 constituyen un conjunto de elementos cruciales en la explicación fisiopatológica de la vaso-oclusión y su relación con diferentes opciones terapéuticas.
Palabras clave: Vaso-oclusión, drepanocitosis, moléculas de adhesión celular, molécula banda 3.
ABSTRACT
The vaso-occlusion in the sickle cell anemia is only characteristic in the haemolytic anemias. The idea that the falciform erythrocyte induces the vaso-occlusive process has been abolished and without doubt the event is produced by the adhesion of the low density deformed erythrocytes ( stress reticulocytes ) to the active vascular endothelium in post-capillary venule participating in the process molecules of cellular adhesion ( erythrocytic and vascular) as well as a group of plasma factors; the external phosphatidilserine , the thrombine action , the expression of tissue factor associated to the disorders of the cationic transportation mechanism as well as the aggregates (band 3) are crucial elements in the pathophysiological explanation of vaso-occlusion and its relation to different therapeutic options.
Key words: Vaso-occlusion,sickle cell anemia, cellular adhesion molecules, band 3 molecule
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References
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