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  • Title: Phenylhydrazine as a partial model for beta-thalassaemia red blood cell hemodynamic properties.
    Author: Ramot Y, Koshkaryev A, Goldfarb A, Yedgar S, Barshtein G.
    Journal: Br J Haematol; 2008 Mar; 140(6):692-700. PubMed ID: 18302715.
    Abstract:
    beta-Thalassaemia is a congenital haemoglobinopathy, associated with red blood cells (RBC) anomalies, leading to impairment of their flow-affecting properties, namely, RBC deformability, self-aggregability, and adherence to endothelial cells (EC). Treatment of normal RBC with phenylhydrazine (PHZ) causes selective association of oxidized alpha-globin chains with the membrane skeleton, leading to reduced RBC deformability, characteristic of beta-thalassaemia. PHZ has thus been used to mimic phenotypes of beta-thalassaemia RBC. The present study was undertaken to further elucidate the suitability of PHZ-treated RBC as a model for beta-thalassemic RBC, by comparing the aggregability and adhesiveness of PHZ-treated RBC to those of RBC from thalassaemia intermedia (TI) patients, using image analysis of RBC under flow. In addition, the externalization of phosphatidylserine (PS), a mediator of RBC/EC interaction, was determined. It was found that PHZ caused enhanced RBC adhesiveness to extracellular matrix, similar to TI-RBC. Furthermore, in both conditions, the enhanced adhesiveness was mediated by PS translocated to the RBC surface. In contrast, PHZ treatment completely abolished RBC aggregability, while TI-RBC aggregability was slightly elevated. It is proposed that PHZ-treated RBC resemble beta-thalassaemia RBC in their deformability and adhesiveness, but not in their aggregability, and thus can be used as a limited model for beta-thalassaemia RBC phenotypes.
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