The incidence of painful crisis in homozygous sickle cell disease:
correlation with red cell deformability
WM Lande, DL Andrews, MR Clark, NV Braham, DM Black, SH Embury and WC Mentzer
Department of Pediatrics, University of California, San Francisco.
To determine whether the vasoocclusive severity of homozygous sickle cell
(SS) disease is influenced by cellular dehydration, we correlated the
incidence of painful crisis with steady-state measurements of red cell
hydration. Sixteen children with SS disease were followed for 3.3 to 8
years (mean, 6.8 years), and a single crisis rate was calculated for each
patient. At the time of well visits, cellular hydration was assessed by
measuring cell deformability, the percentage of red cells with a density
greater than or equal to 1.1056 g/mL, and the percentage of irreversibly
sickled cells (ISC). The incidence of painful crisis showed a strong
positive correlation with Omax, a deformability measurement reflecting
cellular hydration (r = .84, P less than .002), and with hemoglobin
concentration (r = .59, P = .04). That is, higher crisis rates were
observed in patients with less dehydrated, more deformable red cells and
also in patients with higher hemoglobin concentrations. Furthermore, cell
deformability and hemoglobin concentration were independent predictors of
the incidence of painful crisis, which is consistent with separate effects
of these two red cells parameters on vasoocclusive severity.
Volume 72,
Issue 6,
pp. 2056-2059,
12/01/1988
Copyright © 1988 by The American Society of Hematology
