|
|
 Previous Article | Table of Contents | Next Article 
Blood, Vol. 96 No. 3 (August 1), 2000:
pp. 1119-1124
Fetal hemoglobin in sickle cell disease: relationship to
erythrocyte phosphatidylserine exposure and coagulation activation
B. N. Yamaja Setty,
Surekha Kulkarni,
A. Koneti Rao, and
Marie J. Stuart
From the Department of Pediatrics, Division of Research Hematology,
Jefferson Medical College, Thomas Jefferson University; Department of
Medicine and Thrombosis Research, Temple University School of Medicine;
and The Marian Anderson Comprehensive Sickle Cell Center, Philadelphia,
PA.
In sickle cell disease (SCD), loss of erythrocyte membrane
phospholipid asymmetry occurs with the exposure of phosphatidylserine (PS), which provides a docking site for coagulation proteins. In vivo
sickling/desickling, with resulting red cell membrane changes and
microvesicle formation, appears to be one of the factors responsible
for PS exposure. We evaluated children with SCD homozygous for sickle
hemoglobin (SS disease) and controls (n = 65) and
demonstrate that high levels of fetal hemoglobin (assessed as F cells)
are associated with decreased microvesicle formation, PS exposure, and
thrombin generation. F cells correlated inversely with both microvesicles and PS positivity (P < .000001) in
SS disease. Multiple regression analyses using various
hematologic parameters as independent variables, and either
microvesicles or PS positivity as the dependent variable, showed
a strong relationship only with F cells. Additionally, plasma prothrombin fragment F1.2 levels (a marker for
thrombin generation) correlated with both PS positivity
(P < .001) and F cells (P < .01). An F-cell
level of approximately 70% was associated with normal levels of
prothrombin fragment F1.2 and with microvesicle formation
indistinguishable from control values. We suggest that the use of such
surrogate biologic markers in conjunction with F-cell numbers may
provide valuable insights into the biology and consequences of in vivo sickling.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
K. I. Ataga
Hypercoagulability and thrombotic complications in hemolytic anemias
Haematologica,
November 1, 2009;
94(11):
1481 - 1484.
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. Haynes Jr., B. Obiako, R. B. Hester, B. S. Baliga, and T. Stevens
Hydroxyurea attenuates activated neutrophil-mediated sickle erythrocyte membrane phosphatidylserine exposure and adhesion to pulmonary vascular endothelium
Am J Physiol Heart Circ Physiol,
January 1, 2008;
294(1):
H379 - H385.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S.-M. Chung, O.-N. Bae, K.-M. Lim, J.-Y. Noh, M.-Y. Lee, Y.-S. Jung, and J.-H. Chung
Lysophosphatidic Acid Induces Thrombogenic Activity Through Phosphatidylserine Exposure and Procoagulant Microvesicle Generation in Human Erythrocytes
Arterioscler Thromb Vasc Biol,
February 1, 2007;
27(2):
414 - 421.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. I. Ataga and N. S. Key
Hypercoagulability in Sickle Cell Disease: New Approaches to an Old Problem
Hematology,
January 1, 2007;
2007(1):
91 - 96.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. P. Westerman, A. Pizzey, J. Hirshman, M. Cerino, A. Eze, A. Lawrie, G. Purdie, I. Mackie, and J. Porter
Hydroxyurea Treated Sickle Disorders Demonstrate Decreased Plasma Hb, Red Cell Vesicles and Thrombolysis.
Blood (ASH Annual Meeting Abstracts),
November 16, 2005;
106(11):
2322 - 2322.
[Abstract]
|
|
|
|
|
|
|
N. J. Wandersee, S. C. Olson, S. L. Holzhauer, R. G. Hoffmann, J. E. Barker, and C. A. Hillery
Increased erythrocyte adhesion in mice and humans with hereditary spherocytosis and hereditary elliptocytosis
Blood,
January 15, 2004;
103(2):
710 - 716.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
Z. Yasin, S. Witting, M. B. Palascak, C. H. Joiner, D. L. Rucknagel, and R. S. Franco
Phosphatidylserine externalization in sickle red blood cells: associations with cell age, density, and hemoglobin F
Blood,
July 1, 2003;
102(1):
365 - 370.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. N. Y. Setty, S. Kulkarni, and M. J. Stuart
Role of erythrocyte phosphatidylserine in sickle red cell-endothelial adhesion
Blood,
March 1, 2002;
99(5):
1564 - 1571.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. N. Y. Setty, A. K. Rao, and M. J. Stuart
Thrombophilia in sickle cell disease: the red cell connection
Blood,
December 1, 2001;
98(12):
3228 - 3233.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. de Jong, S. K. Larkin, L. A. Styles, R. M. Bookchin, and F. A. Kuypers
Characterization of the phosphatidylserine-exposing subpopulation of sickle cells
Blood,
August 1, 2001;
98(3):
860 - 867.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. N. Y. Setty, S. Kulkarni, C. D. Dampier, and M. J. Stuart
Fetal hemoglobin in sickle cell anemia: relationship to erythrocyte adhesion markers and adhesion
Blood,
May 1, 2001;
97(9):
2568 - 2573.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
