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 Hematoljournal_title
American journal of hematologyauthors
Singer ST,Vichinsky EP,Larkin S,Olivieri N,Sweeters N,Kuypers FA,E\/beta Thalassemia Study Group.doi
10.1002/ajh.21266subject
Has Abstractpub_date
2008-11-01 00:00:00pages
842-5issue
11eissn
0361-8609issn
1096-8652journal_volume
83pub_type
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