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Defective Ca(2+)-induced microvesiculation and deficient expression of
procoagulant activity in erythrocytes from a patient with a bleeding
disorder: a study of the red blood cells of Scott syndrome
EM Bevers, T Wiedmer, P Comfurius, SJ Shattil, HJ Weiss, RF Zwaal and PJ Sims
Department of Biochemistry, Cardiovascular Research Institute Maastricht,
University of Limburg, The Netherlands.
The erythrocytes from a patient with Scott syndrome, a bleeding disorder
characterized by an isolated defect in expression of platelet procoagulant
activity, have been studied. When incubated with the calcium ionophore
A23187, Scott syndrome red blood cells (RBCs) expressed less than 10% of
the prothrombinase (enzyme complex of coagulation factors Va and Xa)
activity of A23187-treated RBCs obtained from normal controls. Consistent
with the results from enzyme assay, the ionophore-treated Scott syndrome
erythrocytes exhibited diminished membrane vesiculation and decreased
exposure of membrane binding sites for factor Va compared with identically
treated controls. When examined by scanning electron microscopy, untreated
Scott syndrome RBCs were indistinguishable from normal cells. After
incubation with A23187, however, the morphology of Scott syndrome RBCs
contrasted markedly from normal erythrocytes. Whereas the Ca2+ ionophore
induced marked echinocytosis and spiculation of normal RBCs, Scott syndrome
RBCs remained mostly discoid under these conditions, with only an
occasional echinocyte-like cell observed. These aberrant responses to
intracellular Ca2+ were also observed for resealed ghosts prepared from
Scott syndrome erythrocytes, indicating that they are related to a defect
in the membrane or membrane-associated cytoskeleton. The finding that the
erythrocytes of this patient share many of the membrane abnormalities
reported previously for Scott syndrome platelets suggests that this defect
is common to both cell lines and involves a membrane component required for
vesicle formation and for expression of prothrombinase sites.
Volume 79,
Issue 2,
pp. 380-388,
01/15/1992
Copyright © 1992 by The American Society of Hematology
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