Hydroxycarbamide-induced changes in E/beta thalassemia red blood cells.

Abstract:

:In thalassemia, fetal hemoglobin (HbF) augmentation with hydroxycarbamide (also known as hydroxyurea) is not always successful. The expected parallel effects on red cell (RBC) membrane deformability, cell hydration, and membrane phospholipid organization, all important for extending RBC life span and increasing Hb, have been infrequently examined. We analyzed these characteristics in 15 nontransfused E/beta(0) thalassemia patients treated with HU (mean 10.2 months). Membrane deformability and cell hydration mildly improved in association with increased HbF levels approaching statistical significance (r = 0.51, P = 0.06). All measures improved considerably in splenctomized patients. These findings underscore the disappointing results of hydroxyurea treatment in clinical trials and the importance of examining the effect on RBC characteristics for the development and understanding of HbF-enhancing agents.

journal_name

Am J Hematol

authors

Singer ST,Vichinsky EP,Larkin S,Olivieri N,Sweeters N,Kuypers FA,E\/beta Thalassemia Study Group.

doi

10.1002/ajh.21266

subject

Has Abstract

pub_date

2008-11-01 00:00:00

pages

842-5

issue

11

eissn

0361-8609

issn

1096-8652

journal_volume

83

pub_type

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