New insights provided by a comparison of impaired deformability with erythrocyte oxidative stress for sickle cell disease.
Blood Cells Mol Dis
; 52(4): 230-5, 2014 Apr.
Article
in En
| MEDLINE
| ID: mdl-24246527
ABSTRACT
Sickle cell disease (SCD) is associated with increase in oxidative stress and irreversible membrane changes that originates from the instability and polymerization of deoxygenated hemoglobin S (HbS). The relationship between erythrocyte membrane changes as assessed by a decrease in deformability and oxidative stress as assessed by an increase in heme degradation was investigated. The erythrocyte deformability and heme degradation for 27 subjects with SCD and 7 with sickle trait were compared with normal healthy adults. Changes in both deformability and heme degradation increased in the order of control to trait to non-crisis SCD to crisis SCD resulting in a very significantly negative correlation between deformability and heme degradation. However, a quantitative analysis of the changes in deformability and heme degradation for these different groups of subjects indicated that sickle trait had a much smaller effect on deformability than on heme degradation, while crisis affects deformability to a greater extent than heme degradation. These findings provide insights into the relative contributions of erythrocyte oxidative stress and membrane damage during the progression of SCD providing a better understanding of the pathophysiology of SCD.
Key words
Full text:
1
Database:
MEDLINE
Main subject:
Oxidative Stress
/
Erythrocyte Deformability
/
Erythrocytes
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Erythrocytes, Abnormal
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Anemia, Sickle Cell
Type of study:
Diagnostic_studies
Limits:
Adolescent
/
Adult
/
Child
/
Female
/
Humans
/
Male
Language:
En
Year:
2014
Type:
Article