Phenylhydrazine as a partial model for β-thalassaemia red blood cell hemodynamic properties

Yuval Ramot, Alexander Koshkaryev, Ada Goldfarb, Saul Yedgar*, Gregory Barshtein

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

25 Scopus citations

Abstract

β-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 α-globin chains with the membrane skeleton, leading to reduced RBC deformability, characteristic of β-thalassaemia. PHZ has thus been used to mimic phenotypes of β-thalassaemia RBC. The present study was undertaken to further elucidate the suitability of PHZ-treated RBC as a model for β-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 β-thalassaemia RBC in their deformability and adhesiveness, but not in their aggregability, and thus can be used as a limited model for β-thalassaemia RBC phenotypes.

Original languageAmerican English
Pages (from-to)692-700
Number of pages9
JournalBritish Journal of Haematology
Volume140
Issue number6
DOIs
StatePublished - Mar 2008

Keywords

  • Adherence
  • Aggregation
  • Phenylhydrazine
  • Phosphatidylserine
  • Thalassaemia

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