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Blood, 1 May 2001, Vol. 97, No. 9, pp. 2568-2573
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Fetal hemoglobin in sickle cell anemia: relationship to
erythrocyte adhesion markers and adhesion
B. N. Yamaja Setty,
Surekha Kulkarni,
Carlton D. Dampier, and
Marie J. Stuart
From the Department of Pediatrics, Division of Research
Hematology, Jefferson Medical College, Thomas Jefferson University;
Division of Hematology, St Christopher's Hospital for Children,
MCP-Hahnemann University School of Medicine; and The Marian Anderson
Comprehensive Sickle Cell Center; all of Philadelphia, PA.
To assess whether fetal hemoglobin (HbF) modulates the
adhesion of sickle erythrocytes to endothelium, children with
homozygous sickle cell anemia (SS disease) were studied, using
this physiologically crucial period to evaluate the relationships
between HbF and the major erythrocyte adhesion markers. The mean level
of CD36+ erythrocytes was 2.59% ± 2.15% (± SD,
n = 40) with an inverse relationship between CD36 positivity and F
cells (R =  0.76, P < .000 00 002). In
univariate analyses, significant correlations with various hematologic
parameters and age were noted. Multiple regression analyses, however,
revealed a relationship solely with F cells. Minimal levels of very
late activation antigen-4+ (VLA4+) erythrocytes
(0.31% ± 0.45%, n = 40) with relationships similar to those
noted for CD36+ cells were also observed. The subpopulation
of strongly adhesive stress reticulocytes was further assessed, using
CD71 as their marker. The mean level of CD71+ erythrocytes
was 5.81% ± 4.21%, with statistical correlates in univariate and
multivariate analyses similar to those discussed above. When adhesion
ratios were evaluated, inverse correlations were noted between basal
and plasma-induced adhesion and F-cell numbers
(R = 0.54, P < .0005;
R = 0.53, P < .0006, n = 39). In
addition, in analyses where basal or plasma-induced adhesion was the
dependent variable and the independent variables included F cells and
the various adhesion-related parameters, significant relationships
solely with F cells were noted. The results demonstrate that SS
patients with higher levels of F cells have concomitant decreases in
the numbers of CD36+, VLA4+, and
CD71+ erythrocytes and that these findings translate into
less adherent erythrocytes. These findings extend knowledge regarding
the protective effects of HbF in the pathophysiology of sickle cell disease.
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