Document Detail

The effect of fetal hemoglobin on the survival characteristics of sickle cells.
MedLine Citation:
PMID:  16861353     Owner:  NLM     Status:  MEDLINE    
The determinants of sickle red blood cell (RBC) life span have not been well-defined but may include both intrinsic factors (eg, the tendency to sickle) and extrinsic factors (eg, the capacity of the reticuloendothelial system to remove defective RBCs). Fetal hemoglobin (HbF) is heterogeneously distributed among sickle RBCs; F cells contain 20% to 25% HbF, whereas the remainder have no detectable HbF (non-F cells). Autologous sickle RBCs were labeled with biotin and reinfused to determine overall survival, non-F- and F-cell survival, and time-dependent changes in HbF content (%HbF) for the surviving F cells. A total of 10 patients were enrolled, including 2 who were studied before and after the percentage of F cells was increased by treatment with hydroxyurea. As expected, F cells survived longer in all subjects. Non-F-cell survival correlated inversely with the percentage of F cells, with the time for 30% cell survival ranging from 6 days in patients with more than 88% F cells to 16 days in patients with less than 16% F cells. As the biotin-labeled RBCs aged in the circulation, the HbF content of the surviving F-cell population increased by 0.28%/d +/- 0.21%/d, indicating that within the F-cell population those with higher HbF content survived longer.
Robert S Franco; Zahida Yasin; Mary B Palascak; Peter Ciraolo; Clinton H Joiner; Donald L Rucknagel
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural    
Journal Detail:
Title:  Blood     Volume:  108     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  2006 Aug 
Date Detail:
Created Date:  2006-07-24     Completed Date:  2006-10-05     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1073-6     Citation Subset:  AIM; IM    
Division of Hematology/Oncology, University of Cincinnati College of Medicine, The Vontz Center for Molecular Studies, 3125 Eden Ave, Ohio 45267-0508, USA.
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MeSH Terms
Anemia, Sickle Cell / pathology*
Cell Survival*
Erythrocyte Aging*
Erythrocytes, Abnormal / pathology*
Fetal Hemoglobin / analysis*
Hemoglobins, Abnormal / analysis
Hydroxyurea / therapeutic use
Time Factors
Grant Support
Reg. No./Substance:
0/Hemoglobins, Abnormal; 127-07-1/Hydroxyurea; 9034-63-3/Fetal Hemoglobin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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