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Hydroxycarbamide‐induced changes in E/beta thalassemia red blood cells
Author(s) -
Singer Sylvia T.,
Vichinsky Elliott P.,
Larkin Sandra,
Olivieri Nancy,
Sweeters Nancy,
Kuypers Frans A.
Publication year - 2008
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.21266
Subject(s) - hydroxycarbamide , beta thalassemia , thalassemia , medicine , hemoglobinopathy , beta (programming language) , hemolytic anemia , chemotherapy , computer science , programming language
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/β 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. Am. J. Hematol., 2008. © 2008 Wiley‐Liss, Inc.