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Rheologic predictors of the severity of the painful sickle cell crisis
SK Ballas, J Larner, ED Smith, S Surrey, E Schwartz and EF Rappaport
Department of Medicine, Thomas Jefferson University, Philadelphia, PA.
Deformable sickle erythrocytes have been reported by Mohandas and Evans to
be more adherent to vascular endothelium than rigid irreversibly sickled
cells (ISC). To define the clinical implications of this finding we have
determined genetic, hematological, clinical, and rheological
characteristics of sickle erythrocytes obtained from 65 patients with
sickle cell anemia and fetal hemoglobin (Hb F) levels less than 15%. The
alpha-globin gene number had a significant effect on the hematological
parameters, the percentage of dense cells, ISC number, and HB A2 levels.
The presence or absence of alpha thalassemia, however, had no effect on the
frequency and severity of the sickle cell painful crisis (r = 0.06, P
greater than .05). RBC deformability, determined by an ektacytometer,
showed great heterogeneity among patients with three or four alpha-globin
genes. Linear regression analyses of the data showed significant positive
correlation of the frequency and severity of the painful crisis with RBC
deformability (r = 0.49, P less than .001), and negative correlations with
the percentage of dense cells (r = -0.37, P = .002), and the percentage of
ISC (r = -0.46, P less than .001). We propose that the more deformable the
sickle RBC are, the greater their adherence to vascular endothelium, and
the more they cause vaso-occlusive crises, RBC deformability and the
percentage of dense cells (or ISC) seem to have a predictive value of the
frequency and severity of painful crises in sickle cell anemia.
Volume 72,
Issue 4,
pp. 1216-1223,
10/01/1988
Copyright © 1988 by The American Society of Hematology
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