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Increased erythrocyte phosphatidylserine exposure in sickle cell disease:
flow-cytometric measurement and clinical associations
BL Wood, DF Gibson and JF Tait
Department of Laboratory Medicine, University of Washington, Seattle
98195-7110, USA.
Increased exposure of phosphatidylserine (PS) in erythrocytes has been
postulated to contribute to the pathophysiology of sickle cell disease
because of its possible effects on blood coagulation, cell adhesion, and
cell clearance. We developed a flow-cytometric assay to measure exposure of
PS on the outer face of the erythrocyte membrane based on addition of
fluorescein-annexin V to whole-blood specimens. The assay correlated
linearly with binding of 125I-annexin V (r2 = .95, n = 125 samples). Normal
donors (n = 30) showed virtually no annexin-positive cells (0.34% +/- 0.18%
for 24-hour old samples). In contrast, annexin V binding was above the
upper limit of normal in 96% of 205 specimens from 17 adult sickle cell and
2 beta-thalassemia patients; the mean percentage of annexin-positive cells
was 2.86% +/- 2.00% (range, 0.4% to 12.0%). Values varied substantially
over time for some patients, and mean values varied between patients. The
percentage of annexin-positive cells always decreased after transfusion (11
events in 6 patients), and out of proportion to the amount of blood
transfused. In conclusion, increased exposure of PS on a subpopulation of
erythrocytes in vivo is a virtually universal feature of sickle cell
disease, and its measurement may be useful to evaluate clinical status and
response to therapeutic measures such as blood transfusion.
Volume 88,
Issue 5,
pp. 1873-1880,
09/01/1996
Copyright © 1996 by The American Society of Hematology
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